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RC341  .T85  A  textbook  of  nervou 


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in  tijr  Cttp  of  31rm  gork 

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Htbrarp 


A    TEXTBOOK 

OF 

NERVOUS    DISEASES 


Digitized  by  the  Internet  Archive 

in  2010  with  funding  from 

Open  Knowledge  Commons 


http://www.archive.org/details/textbookofnervouOOturn 


A    TEXTBOOK 

OF 

NERVOUS  DISEASES 


By 

WILLIAM    ALDREN    TURNER. 

M.D.(Edin.) 

Fellow  of  the  Eoyal  College  of  Physicians  of  London 

Physician  to  King's  College  Hospital  and  Lecturer  in  Neurology 

Physician  to  Out-patients,  National  Hospital  for  the  Paralysed  and  Epileptic 

Queen  Square,  London 

and 

THOMAS    GRAINGER    STEWART 

M.B.(Edin.) 

Member  of  the  Boyal  College  of  Physicians,  London 

Assistant  Physician  to  ths  West  London  Hospital  and  to  the 

National  Hospital  for  the  Paralysed  and  Epileptic,  Queen  Square,  London 


PHILADELPHIA 

P.  BLAKISTON'S  SON  &  CO. 

1012   Walnut  Street 
1910 


134 


Printed  in  Great  Britain 


PREFACE 

The  aim  of  this  book  is  to  provide  the  practitioner  and 
senior  student  with  a  short  and  practical  account  of  the 
Diseases  of  the  Nervous  System.  It  is  not  intended  to  take 
the  place  of  the  larger  and  more  complete  text-books  on 
Nervous  Diseases. 

In  classifying  the  diseases  we  have  adopted  an  arrange- 
ment based  upon  practice  and  personal  experience,  which 
we  hope  may  prove  useful  to  those  for  whom  the  book  is 
primarily  intended. 

Attention  has  especially  been  given  to  the  clinical  de- 
scription of  the  several  disorders — more  particularly  from 
the  point  of  view  of  diagnosis ;  but  the  etiology,  pathology, 
prognosis,  and  treatment  have  each  received  consideration. 

The  limitation  of  the  size  of  the  book  has  made  necessary 
the  omission  of  certain  of  the  disorders  usually  described 
in  treatises  upon  the  nervous  system — such  as  exophthalmic 
goitre,  myxcedema,  and  acromegaly.  For  the  same  reason 
extensive  reference  to  recent  literature  has  been  found 
impossible. 

We  desire  to  record  our  thanks  to  our  colleagues  on  the 
staff  of  the  National  Hospital  for  the  Paralysed  and  Epileptic, 
Queen  Square,  London,  for  permission  to  make  use  of  and 
to  publish  photographs  of  their  cases. 

We  wish  warmly  to  express  our  indebtedness  to  Dr.  Farquhar 
Buzzard  for  his  advice  and  suggestions  in  the   methods    of 


vi  PBEFACE 

classification.  "We  are  also  indebted  to  Dr.  Logan  Turner, 
of  Edinburgh,  for  revising  the  paragraphs  upon  the  exami- 
nation of  the  ear  and  its  diseases. 

Most  of  the  photographs  have  been  prepared  by 
Dr.  S.  A.  K.  "Wilson,  registrar  to  the  hospital ;  but  some 
have  been  given  by  Dr.  Byrom  Bramwell,  Dr.  George  Gibson, 
Dr.  Gordon  Holmes,  and  Dr.  Foster  Kennedy.  Dr.  Gibb 
has  rendered  assistance  in  the  preparation  of  the  index. 

WM.    ALDREN   TURNER, 
T.    GRAINGER   STEWART. 

Harley  Street,  Loxdox,  W. 
Jan.  1910. 


CONTENTS 


Anatomy  and  Physiology 
The  Motor  System     . 
The  Sensory  System 
The  Cerebellar  System 


PART  I 


1 

1 

9 

18 


PART  II 

Examination  or  the  Nervous  System  in  a  Case  of  Nervous 

Disease  ..........  23 

General  Method  and  Scheme  of  Examination    .         .         .  23 
Examination  of  the  Special  Senses  and  the  Cranial 

Nerves     .........  25 

Examination  of  the  Motor  System      .         .         .         .  36 

Examination  of  the  Sensory  System  ....  39 

Examination  of  the  Reflexes        .....  42 

Lumbar  Puncture  :  the  Cerebro- Spinal  Fluid     .  .  48 

Electrical  Examination       .         .        ■*         .         ...  50 

PART  III 

The  Special  Senses 52 

Smell  .         .         .         .         .  .      ■    .         .         .         •  52 

Taste 53 

Yision         ..........  54 

Diseases  of  the  Peripheral  Visual  System  ...  58 

Hearing  and  Equilibration         ......  61 

Diseases   of   the   Eighth   Nerve    and  its   Vestibular 

System 65 

PART  IV 

The    Cranial   Nerves 

The  Oculo-Motor  Nerves "0 

The  Trigeminal  Nerve        ......  80 

The  Facial  Nerve 83 

The  Glossopharyngeal  and  Vagus  Nerves  .... 

The  Hypoglossal  Nerve 

Spinal  Accessory  Nerve     .......  94 

vii  a  3 


Vlll 


CONTENTS 


PART  V 


Tin:    Peripheral  Nervous  System 

Chapter      I.  Lesions  of  the  Peripheral  Nerves 

Lesions  of  Special  Nerves 
Chapter    II.  Paralyses  of  the  Upper  Limb 

1.  Lesions  of  the  Roots  of  the  Brachial 
Plexus        ..... 

2.  Lesions  of  the  Brachial  Plexus 
The  Circumflex  Nerve  . 
The  Musculo- Spiral  Nerve     . 
The  Median  Nerve         .         . 
The  Ulnar  Nerve  .... 

Chapter  III.  Paralyses  of  the  Lower  Limb 

Root  Lesions  .... 

Lesions  of  the  Lumbo-  Sacral  Plexus 

The  Sacral  Plexus 

The  Pudendal  Plexus     . 
Chapter  IV.  Tumours  of  Nerves       .... 

Syphilis  of  the  Peripheral  Nerves 
Chapter     V.  Multiple  Neuritis  ..... 

Alcoholic  Neuritis 

Arsenical  Neuritis  ... 

Lead  Neuritis         .... 

Diabetic  Neuritis  .... 

Post-Diphtheritic  Neuritis     . 

Acute  Toxic  Polyneuritis 

Leprous  Neuritis  .... 

Beriberi  ..... 

Progressive    Hypertrophic    Interstitial 
Neuritis      ..... 
Chapter    VI.  Neuralgia     ...... 

Trigeminal  Neuralgia  (Tic  Douloureux 

Sciatic  Neuralgia — Sciatica  . 

Other  Forms  of  Neuralgia 
Chapter  VII.  Herpes  Zoster 


96 

97 
102 
104 

104 
105 
113 
113 

116 
117 
119 
119 
124 
127 
131 
132 
135 
135 
136 
141 
143 
144 
145 
147 
148 
150 

151 
152 
155 
157 
160 
162 


PART  VI 

The  Diseases  of  the  Brain 

Chapter      I.  The  Intracranial  Blood  Supply     . 
„         II.  General  Symptoms  of  Brain  Disease 
Hemiplegia     .... 
Focal  Epilepsy 
Aphasia,  or  Loss  of  Speech   . 
Apraxia  .... 


166 

166 
171 
173 
177 

180 
186 


CONTENTS 


IX 


The  Diseases  of  the    Brajn — continued. 


Chapter  III. 

Vascular  Lesions  .          .... 

186 

Partial  Occlusion  .... 

187 

Cerebral  Thrombosis 

188 

Cerebral  Embolism 

193 

Cerebral  Hemorrhage    . 

194 

Table  of  Differential  Diagnosis 

204 

Hemiplegia    ...-■.. 

206 

Intracranial  Aneurism  . 

208 

Miliary  Aneurisms 

211 

Chapter  IV. 

Cerebral  Paralyses  of  Infancy  and  Childhoor 

L         211 

Congenital  Cerebral  Diplegia 

212 

Acquired  Cerebral  Paralyses 

216 

Porencephaly          .... 

222 

Atrophic  Sclerosis          ... 

223 

Cerebellar  Affections  in  Children  . 

225 

Chapter     V. 

Intracranial  Tumours  . 

226 

Frontal  Region 

243 

Pre-Central  Region 

246 

Post-Central  and  Parietal  Regions 

250 

Occipital  Lobe        .... 

253 

Temporo- Sphenoidal  Lobe 

254 

Midbrain  and  Subthalamic  Region 

256 

Lateral  Ventricle  .         .         . 

257 

Third  Ventricle 

258 

Falx  Cerebri           .... 

.  ■      259 

Corpus  Callosum 

260 

Pituitary  Body 

261 

Cerebellum    ..... 

262 

Fourth  Ventricle  .         .         .         . 

265 

Cerebello-Pontine  Angle 

267 

Pons  Varolii 

270 

Base  of  the  Skull  .         .         .         . 

272 

Chapter   VI. 

Intracranial  Abscess     .... 

279 

Infective  Sinus  Thrombosis  . 

286 

Chapter  VII. 

Hydrocephalus     .         ... 

288 

PART  VII 


Diseases  of  the  Membranes    . 

Epidemic  Cerebro-  Spinal  Meningitis 
Tuberculous  Meningitis 
Suppurative  Meningitis 


293 

293 
299 

302 


CONTENTS 


PART  VIII  PA0E 

Diseases  of  the  Spinal  Coed 305 

Chapter      I.  Anatomy  and  Physiology  of  the  Spinal  Cord  305 

Relation  of  the  Spinal  Segments    .         .  309 

Localisation  of  Function         .  .  .  311 

Chapter    11.  Intrinsic  Diseases  of  the  Spinal  Cord  .         .  320 

1.  Acute  Myelitis 320 

2.  Cavities  in  the  Spinal  Cord       .         .  327 

3.  Intramedullary  Tumours  .  .  .  336 

4.  Hamiatomyelia  ('  Spinal  Apoplexy  ')  .  340 

5.  Caisson  Disease          ....  344 
Chapter  III.  Diseases  and  Injuries  giving  rise  to  Com- 
pression of  the  Spinal  Cord       .         .         .  346 

1.  Tumours      of      the      Spinal     Mem- 

branes   ......  348 

2.  Caries  of  the  Spine    ....  355 

3.  Malignant  Disease  of  the  Vertebras  .  362 

4.  Traumatic    Lesions    of    the    Spinal 

Column  .  .         .         .         .  364 


PART  IX 

GrENEBAL   DISEASES    OF   THE   NERVOUS    SYSTEM      . 

Chapter      I.   Syphilitic  Diseases        .... 

Cerebro- Spinal  Syphilitic  Lesions 

Syphilitic  Myelitis 

Hypertrophic  Cervical  Pachymeningitis 

Gumma  of  the  Cord 

Syphilitic  Disease  of  the  Bones 
Chapter    II.  Parasyphilitic  Diseases 

1.  Tabes  Dorsalis  .... 

2.  Progressive  General  Paralysis  . 
Chapter  III.  Chronic  Atrophic  Paralyses  of   Spinal  and 

Bulbar  Origin     .... 
Progressive  Muscular  Atrophy 
Amyotrophic  Lateral  Sclerosis 
Chapter  IV.  Disseminated  Sclerosis 

,,  V.   Subacute    Combined    Degeneration    of   the 

Spinal  Cord        ..... 
Chapter   VI.  Acute  Poliomyelitis       .... 
,,        VII.  Landry's  Paralysis        .... 


369 
369 
370 
374 
376 
377 
377 
379 
379 
400 

407 
409 
413 

418 

429 
438 
446 


PART  X 


Vaso-Motoe  and  Trophic  Diseases 
Raynaud's  Disease 
Intermittent  Claudication  . 


449 
449 

452 


CONTENTS 


XI 


VasoMotor  and  Trophic  Diseases — continued. 
Erythromelalgia 
Facial  Hemiatrophy   . 
Angioneurotic  (Edema 
Scleroderma 


PAGE 

454 
156 
457 


PART  XI 


Familial  Diseases 


The  Muscular  Dystrophies 

Amyotonia  Congenita 

Myotonia  Congenita  .... 

Myotonia  Atrophica  .... 

Family  Periodic  Paralysis 

The  Family  Form  of  Muscular  Atrophy  in  Children 

Peroneal  Muscular  Atrophy 

Friedreich's  Disease  .... 

Chronic  Disorders  with  Cerebellar  Sympt 

Hereditary  Spastic  Paraplegia  . 

Amaurotic  Family  Idiocy  . 

Huntington's  Chorea 


458 

458 
467 
469 
470 
470 
471 
472 
474 
478 
481 
482 
483 


Diseases     characterised      by 

Function 

Myasthenia  Gravis    . 
Paramyoclonus  Multiplex 


PART  XII 
Disorders 


of      Muscular 


485 

485 
491 


PART   XIII 

Diseases    oe    Obscure    Origin    characterised    chiefly 
Disorders  of  Motion     . 

Chapter      I.  Paralysis  Agitans 
„         II.  Chorea 
III.  Tetany 
,,        IV.  Occupation  Neuroses 
Tetanoid  Chorea  . 


493 

493 
500 
507 
509 
513 


PART  XIV 
Migraine  and  Periodic  Headache 


515 


PART  XV 


Hysteria  . 

Akinesia  Altera 


522 
546 


XII 


Neuh.vsthknia 


PsYCIIASTHEXIA 


Epilepsy    . 


The  Tics 

Torticollis  Tic    . 

INDEX    . 


CONTENTS 
PART   XVI 

PART   XVII 

PART   XVIII 

PART  XIX 


PAGE 

547 


555 


560 


584 
590 

595 


LIST    OF   ILLUSTRATIONS 


TIG. 
1. 

2. 

3. 
4. 


6. 

7. 

8. 

9. 

10. 

11. 
12. 

13. 
14. 
15. 

16. 
17. 
18. 
19. 

20. 

21a 

21b 

22, 
24. 
25. 
26. 
27. 
28. 
29. 


Photograph  of  the  convexity  of  the  brain  showing  the  motor 

and  sensory  areas         ....... 

Scheme  of  the  convexity  of  the  brain  showing  the  areas 

according  to  Campbell 
The  mesial  surface  of  same 
The  arrangement  and  position  of  the  fibres  in  the  internal 

capsule         ..... 
Diagram   of  the  connexions    of  the   pontine  and   bulbar 

nuclei  ..... 

A  scheme  of  the  sensory  system 
The  thalamocortical  system 
Scheme  of  the  visual  system 
The  cortical  visual  centre    . 
Scheme  of  the  cochlear  and   vestibular    divisions    of  the 

eighth  nerve         ..... 
The  cortical  centres  for  vision  and  hearing 
Paralysis  of  the  left  internal  rectus  muscle 
Paralysis  of  the  right  superior  rectus  muscle 
Paralysis  of  the  right  external  rectus  muscle 
Werner's  '  Artificial  Memory  '  of  the  false  images  in  ocular 

paralyses     ...... 

Complete  paralysis  of  the  left  third  nerve 

Partial  bilateral  third  nerve  paralysis 

Recurring  palsy  of  the  internal  rectus  muscle 

Chart  of  the  sensory  loss  following  excision  of  the  Grasserian 

ganglion       ....... 

Paralysis  of  the  left  fifth  nerve  (motor  division) 
Old  right-sided  facial  paralysis 
Old  facial  palsy  Avith  secondary  over-action 
23.  Bilateral  facial  paralysis      .... 

Facial  palsy  with  herpes  of  the  cervical  plexus 
Unilateral  paralysis  of  the  tongue 
Bilateral  paralysis  of  the  tongue 
Unilateral  paralysis  of  the  tongue 
Paralysis  of  the  spinal  accessory  nerve 
Paralysis  of  serratus  magmas  muscle  (back  view) 


7 
12 
13 
55 
57 

62 
64 
72 
73 
74 

75 
76 

77 
78 

81 
82 
85 
86 
87 
88 
93 
93 
94 
95 
102 


XIV 


LIST  OF  ILLUSTRATIONS 


FIG.  PAGE 

30.  Paralysis  of  serratus  magnus  (side  view)    ....  103 

31.  Area  of  sensory  loss  following  section  of  nerve  roots  from 

the  sixth  cervical  to  the  second  dorsal         .         .         .  105 

32.  Diagram  of  the  brachial  plexus 106 

33.  Sensory  loss  after  lesion  of  the  brachial  plexus  .          .         .  107 

34.  Paralysis  of  the  upper  trunk  of  the  brachial  plexus    .         .  108 

35.  Atrophy  of  the  hand  associated  with  a  cervical  rib     .          .  110 

36.  Sensory   loss   following   lesion    of  the  inner  cord  of  the 

brachial  plexus    .         .         .         .         .         .         .         .  112 

37.  Paralysis  of  the  musculo-spiral  nerve           ....  114* 

38.  Bilateral  paralysis  of  the  same    .         .         .         .         .         .  115 

39.  Sensory  loss  following  lesion  of  the  musculo-spiral  nerve  .  115 

40.  Sensory  loss  following  lesion  of  the  median  nerve       .         .  117 

41.  Paralysis  of  the  ulnar  nerve        .         .         .         .         .         .  118 

42.  Sensory  loss  following  lesion  of  the  ulnar  nerve           .         .  119 

43.  Sensory  loss  after  lesion  of  median  and  ulnar  nerves           .  119 

44.  Three  charts  showing  sensory  loss  from  lesion  of  the  roots 

of  the  cauda  equina      .         .         .         .         .         .         .  121 

45.  Sensory  loss  from  lesion  of  the  roots  from  the  12th  dorsal 

to  the  1st  sacral            .......  122 

46.  Diagram  of  the  lumbo-sacral  plexus    .....  125 

47.  Sensory  loss  following  lesion  of  the  anterior  crural  nerve 

and  its  internal  saphenous  branch        .         .         .         .  127 

48.  Sensory  loss  following  lesion  of  the  great  sciatic  nerve        .  128 

49.  Sensory  loss  following  lesion  of  the  posterior  tibial  nerve  .  130 

50.  Sensory  loss  following  lesion  of  the  external  popliteal  nerve  131 

51.  Drop  feet  of  alcoholic  neuritis     .         .         .         .         .         .  137 

52.  Drop  wrist  of  lead  neuritis           ......  143 

53.  A  case  of  old-standing  sciatica    .         .         .         .         .         .  158 

54.  A  case  of  herpes  zoster       .         .         .         .         .         .         .  164 

55.  Vascular  supply  of  the  convexity  of  the  brain    .         .         .  167 

56.  Vascular  supply  of  the  mesial  surface                  ,  168 

57.  Vascular  supply  of  the  interior  of  the  hemisphere      .         .  169 

58.  Vascular  supply  of  the  interior  of  the  hemisphere     .         .  170 
59-61.  Show  asymmetry  of  the  face  in  a  case  of  incomplete 

right-sided  hemiplegia          ......  175 

62.  A  case  of  cerebral  diplegia          ......  214 

63.  A  case  of  cross-legged  gait           .         .         .         .         .         .  215 

64.  A  case  of  infantile  hemiplegia     ......  219 

65.  A  case  of  athetosis  of  the  hand            .....  220 

66.  A  case  of  athetosis  of  the  toes    ......  220 

67-70.  Various  attitudes  assumed  by  the  hands  in  athetosis    .  221 

71.  A  case  of  atrophic  sclerosis  and  porencephaly     .         .         .  223 

72.  A  tumour  of  the  frontal  lobe      ......  234 

73.  A  tumour  of  the  frontal  lobe      ......  235 

74.  75.  Photographs  showing  the  formation    of    a    '  pressure 

cone' 236,  237 


LIST  OF  ILLUSTRATIONS 


xv 


FIG. 

76. 
77. 
78. 
79. 
80. 
80i 
81. 
82. 
83. 
84. 
85. 
86. 
87. 


89. 

90. 

91. 

92. 

93. 

94. 

95, 

97. 

98. 

99. 
100. 
101. 
102. 
103. 
104. 
105. 

106. 
107. 
108. 
109, 

111. 
112. 

113- 
116. 
117. 
118. 
119. 


PAGE 

A  cystic  glioma  of  the  cerebellum     .....  -■'.'■> 

A  case  of  Jacksonian  epilepsy  ......  247 

Hemorrhage  into  a  subcortical  tumour     ....         248 

A  large  subcortical  tumour       .         .         .         .         .         .  - 1  '• ' 

A  tumour  of  the  choroid  plexus         .         .         .         .         .         257 

.  A  tumour  of  the  third  ventricle       .....  2~>H 

A  tumour  of  the  precuneus       ......         259 

Photograph  showing  '  skew  deviation  '  263 

Photograph  showing  a  cerebellar  attitude  of  the  head  .  264 
Tumour  of  the  right  cerebello -pontine  angle  .  .  .  268 
Bilateral  cerebello -pontine  tumours  .         ....  -<>'■> 

Photograph  showing  Kernig's  sign 295 

Diagram  of  the  tracts,  cell  columns,  and  vascular  supply 

of  the  spinal  cord         .......         306 

Degenerations  descending  from   a   lesion  of  the    spinal 

cord    ..........         307 

Descending  degeneration  following  a  cerebral  lesion 
Front  view  of  body  showing  skin  areas  corresponding  to 

spinal  segments  ...... 

Back  view  showing  the  same    .... 

A  bed-sore  in  the  case  of  acute  myelitis    . 

Sensory  loss  in  a  case  of  meningo-myelitis 

Suppurative  myelitis  (spinal  abscess)        ; 

96.  Paralysis  of  the  cervical  sympathetic  nerve 

Atrophy  of  the  hand  and  arm  in  syringomyelia 

Wasting  of  muscles  in  syringomyelia 

Sensory  loss  in  syringomyelia  . 

'  Pincer  hand  '  in  syringomyelia 

'  Succulent  hand '  in  syringomyelia  . 

Painless  whitlows  in  syringomyelia  . 

Photograph  of  a  large  intramedullary  tumour 

Photograph  of  cavities  in  the  spinal  cord 

Sensory  loss  in  a  case  of  intramedullary  tumour  in  the 

cervical  region     ...... 

Position  of  the  arms  in  a  case  of  hasmatomyelia 

Sensory  loss  in  a  case  of  hsematomyelia    . 

'  Preacher's  hand '  in  a  case  of  haamatoinyelia  . 

110.  Photographs    of    two    cases    of    intrathecal    spinal 

tumours       ..... 
Sensory  loss  in  a  case  of  spinal  tumour 
Photograph  showing  paralysis  of  the  lower  part  of  the 

recti  abdominales 
115.  Spinal  cord  in  a  case  of  tabes  dorsalis 
Photograph  of  the  tabetic  face  . 
Photograph  showing  genu  recurvatum 
Photograph  showing  hypotonia 
Sensory  loss  in  a  case  of  tabes  dorsalis     . 


316 
317 
322 
323 
326 
330 
331 
332 
333 
334 
334 
335 
337 
338 

339 
341 
342 
343 

349 
352 

358 
382 
387 
389 
390 
392 


XVI 


LIST  OF  ILLUSTRATIONS 


FIG.  PAGE 

120.  Photograph  of  a  Charcot  arthropathy        ....  394 

121.  Photograph  of  perforating  ulcers  .....  395 
122-124.  Spinal  cord  in  amyotrophic  lateral  sclerosis  .  .  408 
125.  Hands  in  progressive  muscular  atrophy  ....  409 
1:26.  Arms  and  attitude  in  progressive  muscular  atrophy  .  410 
1:27.  Hands  in  amyotrophic  lateral  sclerosis      ....  413 

128.  Hands  in  amyotrophic  lateral  sclerosis      ....  414 

129,  130.  The  face  and  tongue  in  bulbar  paralysis    .          .         .  415 

131.  The  face  in  chronic  ophthalmoplegia         ....  416 

132,  133.   Spinal  cord  in  disseminated  sclerosis          .         .          .  419 

134.  Photograph  of  hyper-extension  of  the  toes  in  a  case  of 

disseminated  sclerosis          ......  425 

135.  Spinal  cord  in  subacute  combined  sclerosis       .         .         .  430 
136-139.   Spinal  cord  in  subacute  combined  sclerosis        .          431,  432 
140.   Spinal  cord  in  subacute  combined  sclerosis       .          .         .  433 
141-143.  Charts   illustrating  the  sensory  symptoms    in  sub- 
acute combined  sclerosis     ......  435 

144.  A  case  of  extensive  acute  poliomyelitis     ....  440 

145.  Acute  poliomyelitis  affecting  the  abdominal  muscles         .  441 

146.  Wasting  of  arm  in  an  old  case  of  poliomyelitis          .          .  442 

147.  Talipes  valgus  in  poliomyelitis           .....  443 

148.  Talipes  calcaneus  in  poliomyelitis      .....  444 

149.  Spinal  curvature  in  poliomyelitis      .....  445 

150.  A  case  of  facial  hemiatrophy     ......  455 

151.  152.  Cases  of  pseudo-hypertrophic  muscular  paralysis      .  461 

153.  A  case  of  Erb's  juvenile  muscular  atrophy        .          .         .  463 

154.  Photograph  of  the  face  in  the  facio-scapulo-humeral  type  464 

155.  Photograph  of  the  myopathic  face    .....  465 

156.  Erb's  juvenile  muscular  atrophy       ......  467 

157.  158.  A  case  of  amyotonia  congenita  .....  468 

159.  A  case  of  peroneal  muscular  atrophy         .         .          .         .  472 

160.  A  case  of  peroneal  muscular  atrophy         ....  473 

161.  The  feet  in  Friedreich's  disease         ......  475 

162.  The  scoliosis  of  Friedreich's  disease          ....  476 

163.  164.  A  case  of  family  spastic  paralysis      ....  481 

165.  Photograph  of  ophthalmoplegia  externa  in  myasthenia 

gravis  . 

166.  The  myasthenic  smile 

167.  The  face  in  paralysis  agitans    . 

168.  The  attitude  in  paralysis  agitans 

169.  170.  The  attitude  in  paralysis  agitans 

171.  The  hands  in  paralysis  agitans 

172.  The  hands  in  chorea 

173.  Hysterical  spasm  of  the  face 

174.  Hysterical  palsy  of  the  arm 

175.  Hysterical  drop  wrist 

176.  Hysterical  paraplegia 


487 
487 
495 
496 
497 
498 
504 
529 
530 
531 
532 


LIST  OF  ILLUSTKATIONS 


XVJl 


177.  Hysterical  contracture  of  hand  and  fingers 

178.  Hysterical  contracture  of  ankle  and  foot  . 

179.  Hysterical  contracture  of  ankle  and  foot  . 

180.  181.  Charts  showing-  hysterical  anaesthesia 

182.  Charts  showing  hysterical  hemianesthesia 

183.  Hysterical  contracture  of  hip  joint  . 

184.  185.  Photographs  of  two  cases  of  tic  movements  of  the 

head  and  neck     ...... 

186.  Tic-like  spasm  of  the  face         .... 

187.  A  case  of  torticollis  tic     . 

188.  A  case  of  antero-collic  spasm    .... 


PAGE 

533 
533 
534 
536 
537 
541 

585 
587 
591 
592 


NERVOUS    DISEASES 


Part  I 

ANATOMY  AND   PHYSIOLOGY 

OF  THE  MOTOR,  SENSORY,  AND  CEREBELLAR 
SYSTEMS 


THE  MOTOR  SYSTEM 

The  motor  system  consists  of  the  cortical  centres,  the 
efferent  cortical  or  pyramidal  fibres,  the  bulbo-spinal  centres, 
and  the  motor  fibres  of  the  peripheral  nerves.  By  means  of 
this  system  voluntary  impulses,  emanating  from  the  cerebral 
cortex,  are  conveyed  to  the  muscles.  It  may  be  described 
under  two  sub-systems  :  — 

1.  The  upper,  cortico-spinal  or  pyramidal  system. 

2.  The  lower,  spino-muscular  or  peripheral  system. 

i.  The  pyramidal  system  (upper  motor  neurone) 

The  cortical  motor  areas.  In  the  higher  apes  and  man, 
the  motor  area  lies  in  front  of  the  fissure  of  Rolando,  and 
occupies  the  precentral,  or  ascending  frontal  convolution  and 
the  anterior  wall  of  the  fissure.  The  centres  for  the  move- 
ments of  the  trunk  and  limbs  are  situated  on  the  outer  aspect 
of  the  cerebrum  from  above  downwards,  as  follows  :  foot,  leg, 
hip,  trunk,  shoulder,  arm,  hand,  face,  lips,  and  tongue. 
Upon  the  mesial  aspect  the  centre  for  the  leg  extends  into 
the  paracentral  lobule.     (Fig.  1.) 

The  motor  or  precentral  area  is  characterised  by  the 
presence  of  large  pyramidal  cells  and  of  giant  cells  (cells  of 
Betz)  throughout  its  whole  extent,  with  the  exception  of  that 
portion  which  corresponds  to  the    'face   area'    (Campbell). 


2  NERVOUS  DISEASES 

Immediately  in  front  of  the  precentral  area  and  correspond- 
ing to  the  posterior  ends  of  the  first,  second,  and  third  frontal 
gyri,  and  a  considerable  part  of  the  marginal  gyrus,  is  the 
'  intermediate  precentral  area '  of  Campbell.1  In  structure  it 
resembles  the  precentral  area,  but  contains  fewer  nerve  fibres 
and  no  Betz  cells.     (Figs.  2  and  3.) 

The  precentral  area  is  that  portion  of  the  cerebral  cortex 
in  which  crude  voluntary  movements  of  the  body  and  limbs 
are  represented.  Its  electrical  stimulation  is  followed  by  clonic 
convulsions  of  the  body  and  limbs  on  the  opposite  side.     The 


Fig.  1. — The  convexity  of  the  left  cerebral  hemisphere,  showing  the  motor 
area  (shaded)  in  the  front  of  the  fissure  of  Rolando  and  the  sensory  area 
of  the  post-central  convolution. 

'intermediate  precentral,'  or  higher  motor  (psychomotor)  area 
is  regarded  as  presiding  over  more  specialised  or  skilled  move- 
ments than  the  precentral  zone,  as  in  it  are  contained  the 
centres  for  articulatory  speech  and  writing. 

The  pyramids  or  pyramidal  tracts  originate  in  the  grey 
cortex  of  the  precentral  area,  and  are  composed  of  fibres 
arising  from  the  large  giant  cells  of  that  area.  These  fibres 
descend  through  the  centrum  ovale,  form  a  portion  of  the 
corona  radiata  and  reach  the  internal  capsule,  where  they 

1    Campbell,  Localisation  of  Cerebral  Function,  1905. 


ANATOMY  AND  PHYSIOLOGY 


3 


occupy  a  position  partly  at,  and  partly  behind,  the  '  knee '  of 
that  structure.  The  arrangement  of  the  fibres  in  the  internal 
capsule  is  such  that  those  which  come  from  the  upper  cortical 
areas  lie  posteriorly,  and  those  from  the  lower  areas  anteriorly. 


Pig.  2. — The  motor  and  sensory  areas  of  the  convexity  of  the  brain  according 
to  Campbell,  r,  the  fissure  of  Rolando.  A,  intermediate  precentral  area. 
B,  precentral  area.  C,  post-central  area.  D,  intermediate  post-central 
area.     E,  parietal  area. 


Fig.  3. — The  motor  and  sensory  areas  upon  the  mesial  surface  of  the  brain, 
according  to  Campbell.     The  lettering  indicates  the  same  area  as  in  fig.  2. 

Anterior  to,  and  in  the  region  of  the  'knee '  are  the  fibres  for 
the  eyes,  head,  tongue,  and  face ;  in  the  posterior  limb  from 
before  backwards  are  those  for  the  shoulder,  arm,  hand,  trunk, 
hip,  leg,  and  foot.  The  fibres  for  the  leg  lie  close  to  those  of 
common  and  special  sensation  in  the  retrolenticular  portion 

*  i 


NKHVOrs   DISK  ASKS 


of  the  internal  capsule.  From  the  internal  capsule  the 
pyramidal  fibres  pass  downwards  into  the  pes  pedunculi,  and 

onwards  through  the  ventral  region  of  the  pons  Varolii  to 
form  the  pyramids  of  the  medulla  oblongata.  On  reaching 
the  bulb  the  two  pyramids  become  more  closely  approximated 
than  in  any  previous  part  of  their  course. 

During  their  progress 
through  the  mid  and  hind 
brain  a  great  diminution 
in  the  size  of  the  pyramids 
takes  place,  owing  to  a 
large  number  of  fibres 
being  given  off  to  the  motor 
cranial  nerve  nuclei,  and 
to  other  structures,  as 
follows  : — 

(a)  To  the  third  and 
sixth  nuclei.  It  is  not 
exactly  known  how  these 
nuclei  receive  pyramidal 
fibres,  whether  by  special 
bundles  or  by  means  of 
scattered  fibres. 

(b)  To  the  motor  trige- 
minal, facial,  and  hypo- 
glossal nuclei,  by  the 
'  accessory  fillet.' 

(c)  To  the  nucleus  am- 
biguus,  or  motor  vago- 
glossopharyngeal nucleus, 
by  '  Pick's  bundle.' 

(d)  A  ventro-lateral  pyramidal  bundle  has  been  described 
(Barnes),  but  it  is  doubtful  whether  it  contains  pyramidal  or 
other  descending  fibres. 

(e)  Thalamo-spinal,  rubro-spinal,  and  ponto-spinal  bundles. 
The  existence  of  these  tracts  may  explain  the  retention  of 
some  movement  when  the  pyramids  are  completely  severed, 
the  restitution  of  function  after  cerebral  lesions  with  degenera- 
tion of  the  pyramidal  tracts  and  the  continuance  of  artificially 
induced  epileptic  convulsions  after  section  of  the  pyramids. 

The  great  majority  of  the  pyramidal  fibres   cross    at    the 


Pig.  !  (from Cunningham's  'Anatomy'). 
Shows  the  arrangemenl   and  position 
of  the  afferent  and   effereni   fibres  in 
the  interna]  capsule. 


ANATOMY  AND  PHYSIOLOGY  5 

decussation  of  the  pyramids  to  form  the  lateral,  or  crossed 
pyramidal  tract  of  the  spinal  cord.  A  relatively  small  number 
pass  downwards  in  the  antero-internal  region  of  the  same 
side,  as  the  direct  pyramidal  tract,  and  decussate,  by  way  of  the 
anterior  commissure  at  succeeding  levels,  into  the  grey  horns 
of  the  opposite  side. 

The  fibres  of  the  crossed  pyramidal  tract  give  off  collaterals 
into  the  grey  matter  throughout  the  whole  length  of  the 
spinal  cord,  and  the  tract  may  be  traced  as  a  diminishing 
structure  as  far  down  as  the  fourth  sacral  segment.  The 
arborescence  of  the  pyramidal  fibres  round  the  anterior 
cornual  cells  has  not  yet  been  demonstrated,  and,  according 
to  some  observers,  they  terminate  in  relation  to  the  cells 
of  the  posterior  horns. 

By  means  of  the  pyramidal  fibres,  the  motor  cortex  of  the 
precentral  area  is  brought  into  relation  with  the  bulbo-spinal 
centres  and  nuclei  of  all  segments  of  the  limbs  and  body  from 
the  eyes  to  the  perinseum. 

Owing  to  the  complete  decussation  of  the  motor  fibres,  a 
destructive  lesion  of  one  cerebral  hemisphere,  involving  the 
pyramidal  system,  is  followed  by  paralysis  of  the  movements 
of  the  opposite  side  of  the  body. 

2.  The  peripheral  motor  system  (lower  motor  neurone) 

The  peripheral  motor  system  consists  of  the  bulbo-spinal 
centres,  their  issuing  motor  roots,  and  the  motor  fibres  of  the 
peripheral  nerves. 

The  bulbo-spinal  centres  are  collected  into  groups  of 
ganglion  cells.  In  the  hind  brain  these  collections  of  cells 
form  the  nuclei  of  the  motor  cranial  nerves,  and  give  origin 
to  their  respective  motor  nerves,  or  to  the  motor  fibres  of  the 
mixed  cranial  nerves.  In  the  spinal  cord  the  separation  of 
the  centres  is  less  defined,  although  two  distinct  enlargements 
are  seen  corresponding  to  the  upper  and  lower  limbs. 

The  following  is  a  brief  account  of  the  motor  nuclei  of  the 
cranial  nerves  and  their  homologues  in  the  anterior  horns. 

The  third  nucleus  consists  of  an  elongated  group  of  nerve 
cells  lying  in  the  grey  matter  of  the  floor  of  the  aqueduct  of 
Sylvius,  which  connects  the  third  and  fourth  ventricles.  The 
nucleus  has  a  segmentary  character,  the  several  groups  of  cells 


6  NERVOUS  DISEASES 

corresponding  to  separate  ocular  muscles,  or  groups  of  muscles. 
Quite  at  its  posterior  end  is  a  group  of  cells  which  would 
seem  to  innervate  the  levator  palpebral  superioris,  the  median 
groups  probably  supply  the  internal  and  inferior  recti  muscles, 
and  the  lateral  group  the  superior  rectus  and  inferior  oblique 
muscles  of  the  eye.  There  is  also  an  antero-lateral  group  of 
small  cells,  which  was  at  one  time  supposed  to  supply  the 
pupillary  fibres  and  those  for  the  ciliary  muscle.  The  roots 
of  the  third  nerve  pass  through  the  tegment  of  the  crus  and 
issue  mesially  to  the  pes  cruris  at  the  upper  border  of  the 
pons  Varolii,  and  proceed  into  the  orbit  through  the  sphenoidal 
fissure. 

The  fourth  nucleus  is  situated  posterior  to  the  third 
nucleus,  of  which  it  is  really  the  distal  continuation.  The 
nerve  roots  decussate  after  they  have  left  the  nucleus  in  the 
superior  medullary  velum  and  pass  forwards  into  the  orbit  to 
supply  the  superior  oblique  muscle. 

The  sixth  nucleus  lies  some  distance  behind  the  above  in 
the  dorsal  portion  of  the  tegmentum  pontis,  under  the  floor 
of  the  fourth  ventricle.  Its  nerve  root  passes  ventrally 
through  the  tegment  of  the  pons  to  issue  at  its  lower  margin. 
It  passes  forwards  to  enter  the  orbit  and  to  supply  the  ex- 
ternal rectus  muscle.  In  the  tegmentum  pontis  the  sixth 
nucleus  is  enveloped  by  the  issuing  root  of  the  seventh  nerve. 

The  oculo-motor  nuclei  are  connected  with  each  other 
and  with  the  spinal  cord  by  fibres  of  the  posterior  longitudinal 
bundle.  In  this  bundle  are  fibres  which  connect  the  sixth 
nucleus  of  one  side  with  the  opposite  third  nucleus,  a  con- 
nexion which  subserves  the  function  of  conjugate  movement  of 
the  eyeballs.  The  sixth  nucleus  is,  therefore,  not  only  the 
motor  nucleus  for  the  homo-lateral  external  rectus  muscle, 
but  is  also  the  centre  for  the  associated  action  of  the  external 
and  internal  recti  of  opposite  sides. 

The  motor  nucleus  of  the  fifth  nerve  is  formed  partly  by 
a  group  of  large  cells  situated  in  the  lateral  part  of  the 
tegmentum  pontis,  and  partly  by  a  column  of  cells  situated  in 
the  Sylvian  grey  matter — the  so-called  trophic  or  descending 
trigeminal  root.  Both  these  sets  of  cells  give  fibres  to  the 
motor  root,  which  is  distributed  mainly  to  the  muscles  of 
mastication.     (Fig.  5.) 

The  seventh  nucleus  is  a  segmented  nucleus  occupying  a 


ANATOMY  AND  PHYSIOLOGY  7 

ventral  position  in  the  tegmentum  pontis.  The  nerve  root 
passes  dorsally  towards  the  floor  of  the  fourth  ventricle,  and, 
turning  round  the  sixth  nucleus,  courses  through  the  teg- 
mentum and  issues  on  the  ventro-lateral  aspect  of  the  pons. 
It  supplies  the  facial  muscles  of  expression.     (Fig.  5.) 


Fig.  5  (from  Cunningham's  'Anatomy'). — Shows  the  connexions  of  the  vagal, 
glossopharyngeal,  auditory,  facial,  abducent,  and  trigeminal  nerves. 


The  nucleus  ambiguus  is  the  motor  nucleus  for  the  com- 
bined vagoglossopharyngeal  nerve.  It  is  a  long  nucleus 
lying  in  the  reticular  formation  of  the  medulla  oblongata.  Its 
nerve  roots  form  the  motor  fibres  of  the  glossopharyngeal  and 
vagus  nerves.     (Fig.  5.) 


8  NERVOUS  DISEASES 

The  twelfth,  nucleus  is  a  segmented  nucleus  lying  in  the 
dorsal  portion  of  the  medulla,  and  is  rich  in  medullated  nerve 
hbres.  .Its  roots  pass  through  the  reticular  formation  and 
issue  on  the  anterior  surface  of  the  bulb  external  to  the 
pyramids.      (Fig.  5.) 

The  nuclei  of  the  anterior  horns  presiding  over  the  muscles 
of  the  head  and  neck,  trunk  and  limbs,  are  situated  in  the 
anterior  horns  throughout  the  whole  extent  of  the  spinal 
cord. 

According  to  the  description  given  by  Lenhossek,  the  most 
mesially  placed  group  of  cells  in  the  anterior  cornual  region  is 
commissural,  not  motor  in  function.  The  motor  cells  lie 
external  to  the  mesial  group.  In  the  upper  cervical  and 
dorsal  regions  of  the  cord,  the  motor  cells  are  grouped  into 
a  single  nucleus  :  in  some  places  slightly  separated  from,  in 
others  in  juxtaposition  to,  the  mesial  or  commissural  group. 
In  the  cervical  and  lumbar  enlargements  on  the  other  hand, 
the  motor  cells  become  separated  into  two  large  and  well- 
defined  nuclei,  having  an  antero-lateral  and  postero-lateral 
position  respectively  in  the  anterior  horn.  In  the  lower 
lumbar  and  sacral  regions  a  third  group  has  been  described 
occupying  a  central  position. 

The  nucleus  in  the  intermedio-lateral  horn,  which  is  present 
between  the  eighth  cervical  and  second  lumbar  roots,  has 
been  found  by  Bruce1  to  be  connected  with  the  sympathetic 
system. 

Of  the  fibres  forming  the  anterior  roots,  some  are  coarse 
and  others  are  fine.  It  is  generally  supposed  that  the  fibres 
of  larger  calibre  pass  to  the  voluntary  muscles,  while  the  finer 
are  destined  for  the  sympathetic  system  by  way  of  the  rami 
communicantes. 

The  general  conclusions  which  may  be  derived  from 
numerous  studies  upon  the  functions  of  the  motor  nuclei  in 
the  anterior  horns  are,  first,  that  muscles  and  not  move- 
ments are  represented  in  them ;  and  secondly,  that  each 
segment  of  the  cord  presides  over  portions  of  a  number  of 
different  muscles.  (For  further  details  on  this  subject,  the 
reader  is  referred  to  p.  317.) 

1  Bruce,  Review  of  Neurology,  1907. 


ANATOMY  AND  PHYSIOLOGY         0 

THE  SENSORY  SYSTEM 

Anatomy  <>v  the  Sknsouy  Systkm 

For  purposes  of  description  the  sensory  system  may  be 
divided  into  three  sub-systems  :  — 

1.  The  lowest,  or  system  of  the  posterior  root  ganglion, 
includes  the  sensory  end-organs,  the  sensory  fibres  of  the  peri- 
pheral nerves,  and  the  posterior  roots  with  their  extensions 
into  the  spinal  cord.  It  also  includes  the  sensory  cranial 
nerves,  and  their  bulbar  continuations. 

2.  The  middle  or  spino-thalamic  systems  of  the  cord  and 
the  cranial  nerves. 

3.  The  highest,  or  thalamocortical  system. 

The  posterior  ganglionic  system  consists  of  nerve  fibres, 
which  have  their  trophic  cells  in  the  ganglia  of  the  posterior 
spinal  roots,  or  their  cranial  homologues — Gasserian,  genicu- 
late, jugular,  petrosal,  and  vagal. 

The  sensory  fibres  pass  at  the  inter-vertebral  foramina 
into  the  posterior  nerve  roots  of  their  respective  segments. 

The  central  terminations  of  the  spinal  and  cranial  sensory 
roots  will  be  separately  considered. 

(1)  Spinal.  The  fibres  of  the  posterior  nerve  roots  are 
primarily  divided  into  two  sets,  fine  and  coarse  fibres.  The 
fine  fibres  enter  the  posterior  horn  as  the  zone  of  Lissauer. 
The  coarse  fibres  take  up  a  position  along  the  posterior  horn 
of  grey  matter  (the  cornu-radicular  zone  of  Marie) .  This  zone 
is  little  by  little  displaced  in  a  mesial  direction  by  the  entrance 
of  other  nerve  roots  at  successively  higher  levels.  The  desti- 
nation of  the  coarse  fibres  is  as  follows  :  (a)  by  short  fibres 
terminating  in  the  posterior  horn  of  the  same  side ;  (b)  by 
medium  fibres  passing  some  distance  up  the  posterior  columns, 
and  terminating  in  Clarke's  group  of  cells ;  and  (c)  by  long 
fibres  which  pass  up  the  posterior  columns  to  the  posterior 
columnar  nuclei.  On  their  passage  upwards  in  the  posterior 
columns,  the  long  fibres,  entering  from  the  lumbo-sacral  and 
lower  dorsal  nerve  roots,  occupy  the  postero-median  column  : 
those  from  the  upper  dorsal  and  cervical  roots  ascend  in  the 
postero- external  column. 

(2)  Cranial.     The  afferent   fibres    of  the  sensory  cranial 


10  NERVOUS  DISEASES 

nerves  are  arranged  in  a  somewhat  different  manner,  being 
collected  into  definite  bundles. 

(a)  The  trigeminal  nerve.  The  centripetal  fibres  of  the 
Gasserian  ganglion  on  reaching  the  pons  are  collected  into 
the  spinal  root  of  the  trigeminus,  which  passes  distally  as  far 
as  the  second  cervical  nerve.  The  fibres  of  this  root  terminate 
in  the  gelatinous  substance  of  Rolando  which  lies  upon  its 
mesial  aspect.     (Fig.  5.) 

(b)  The  sensory  division  of  the  facial  nerve,  or  nerve  of 
"Wrisberg  (pars  intermedia),  enters  the  medulla  in  association 
with  the  internal  root  of  the  auditoiy  nerve.  In  a  case 
observed  by  Eamsay  Hunt  1  the  fibres  terminated  in  the 
fasciculus  solitarius. 

(c)  The  vagoglossopharyngeal  nerve.  The  afferent  fibres 
of  this  nerve  divide  into  two  series,  one  of  which  forms  the 
fasciculus  solitarius,  a  structure  analogous  to  the  spinal  root 
of  the  trigeminus,  and  the  other  terminates  in  the  posterior 
vago-glossopharyngeal  nucleus.  The  fibres  of  the  fasciculus 
solitarius  end  in  the  contiguous  gelatinous  substance.    (Fig.  5.) 

Sensory  impressions  are  conveyed  from  the  spinal  cord 
to  the  brain  by  (a)  the  mesial  fillet;  (b)  the  spino-thalamic 
system. 

The  mesial  fillet.  From  the  posterior  columnar  nuclei,  in 
which  the  posterior  columns  of  the  spinal  cord  terminate,  an 
extensive  decussation  takes  place  into  the  opposite  inter- 
olivary  layer.  The  mesial  fillet  emerges  from  this  layer,  and, 
passing  through  the  medulla  and  pons,  terminates  in  the 
ventro-lateral  region  of  the  optic  thalamus.  It  conveys  the 
impressions  transmitted  by  the  posterior  columns  in  a  cerebral 
direction. 

The  spino-thalamic  system.  This  would  appear  to  be 
the  path  by  wdiich  some  of  the  entering  posterior  root  fibres 
are  indirectly  continued  towards  the  brain.  Fibres,  arising 
in  cells  situated  in  the  posterior  horns,  cross  to  the  opposite 
side  by  the  posterior  commissure  and  ascend  in  the  antero- 
lateral column  of  the  spinal  cord,  where  they  form  a  series  of 
short  systems  of  ascending  fibres,  lying  mesial  and  ventral  to 
Gowers's  tract.  These  fibres  ascend  through  the  spinal 
cord,  medulla,  and  pons  into  the  ventral  portions  of  the  optic 
thalamus.     In  the  medulla  and  pons  they  receive  accessions 

1  Eamsay  Hunt,  Journ.  New.  and  Mental  Disease,  1907. 


ANATOMY  AND  PHYSIOLOGY  11 

of  fibres  from  the  sensory  cranial  nerves,  more  especially  the 
trigeminus  of  the  opposite  side. 

The  distribution  of  the  sensory  fibres  would  appear  to  be 
as  follows : — 

1.  Some  of  the  fibres  are  continued  for  a  considerable 
distance  upon  the  same  side  as  the  entering  sensory  nerves, 
either  as  the  posterior  columns  of  the  cord,  or  as  the  descending 
spinal  root  of  the  trigeminus  and  the  vagoglossopharyngeal 
nerves.     (Fig.  6  (1).) 

2.  Others  are  continued  for  a  longer  or  shorter  distance 
upon  the  same  side  prior  to  their  decussation,  as  it  is  obvious, 
on  the  one  hand,  that  the  posterior  columns  in  the  cervical 
region  cannot  contain  all  the  fibres  which  have  passed  into 
them  from  the  lower  segments,  and,  on  the  other  hand,  the 
spinal  roots  of  the  trigeminus  and  vago-glossopharyngeus  are 
structures  which  diminish  in  size  as  they  approach  their 
termination.     (Fig.  6  (2).) 

3.  Other  fibres  decussate  at  once  on  entering  the  spinal 
cord  or  the  bulb.     (Fig.  6  (3).) 

Whatever  is  the  immediate  disposition  of  the  entering 
sensory  fibres,  whether  spinal  or  cranial,  it  is  clear  that  they 
all  decussate  before  they  reach  the  upper  portion  of  the  hind 
brain. 

The  thalamo-cortical  system.  A  great  mass  of  fibres 
radiates  in  a  cortical  direction  from  the  optic  thalamus 
and  passes  to  most  regions  of  the  cerebral  cortex  both 
of  the  same  and  of  the  opposite  side,  reaching  the  latter 
through  the  corpus  callosum.  Which  portion  of  this 
thalamic  projection  system  belongs  to  the  afferent  or  sensory 
pathway  under  discussion  is  uncertain  ;  but  it  would  seem 
possible  to  differentiate  two  primary  systems,  which  medullate 
at  different  periods  (Flechsig)  :  (a)  A  tract  of  fibres,  medul- 
lating  at  the  ninth  month,  passes  to  the  post-central  gyrus. 
This  is  probably  the  cortical  termination  of  the  mesial  fillet. 
(b)  A  tract,  medullating  during  the  first  month  of  extra-uterine 
life,  passes  to  the  falciform  lobe.  These  fibres  probably 
occupy  the  retrolenticular  portion  of  the  internal  capsule  in 
close  relation  to  the  fibres  of  the  adjacent  optic  radiation. 
(Fig.  7.) 

The  cortical  centres  for  common  sensation  will  be  con- 
sidered more  particularly  under  the  physiology  of  the  sensory 


12 


NERVOUS  DISEASES 


/w, 


F.S. 


P.R.Z 


Fig.  6. — Scheme  of  the  sensory  system  in  the  spinal  cord,  and  brain.  P.R., 
posterior  nerve  roots.  1,  direct  fibres  of  posterior  columns.  2,  fibres 
which  pass  for  a  short  distance  in  the  posterior  columns.  3,  the 
decussating  fibres.  Med.,  medulla  oblongata.  5th,  trigeminal  nerve.  0.  th., 
optic  thalmus.  C.C.,  'corpus  callosum.  L.N.,  lenticular  nucleus.  F.S., 
Sylvian  fissure. 


ANATOMY  AND  PHYSIOLOGY 


13 


system  (p.  18),  but  it  has  been  shown  by  Campbell  that  the 
post-central  gyrus  is  structurally  in  harmony  with  certain 
other  cortical  areas,  which  are  known  to  be  sensory  in  function. 
The  special  feature  of  this  zone  is  the  presence  of  a  plexus 
of  nerve  fibres  of  large  calibre,  having  an  oblique  course  and 
running  at  right  angles  to  the  radiating  fasciculi. 


CALLOSUM 


T»  LIMB 
T'rCAPSULE 


LOBE 


Fig.  7  (from  Cunningham's  '  Anatomy  '). — Shows  the  afferent  or 
thalamocortical  projection  system  of  fibres. 


Physiology  op  the  Sensoky  System 

Our  conception  of  the  physiology  of  the  sensory  system 
has  been  enlarged  by  the  recent  observations  of  Head,1  the 
general  results  of  whose  work  have  been  embodied  in  the 
following  account. 

1  Head,  Brain,  1905  -1906. 


14  NERVOUS  DISEASES 


The  peripheral  nervous  system 

Head  has  shown  that  in  the  peripheral  nervous  system 
there  is  a  physiological  grouping  of  the  various  forms  of 
sensibility. 

1.  Epicritic  sensibility,  consisting  of  the  appreciation  of 
light  touch,  the  finer  grades  of  temperature,  cutaneous  localisa- 
tion, and  the  discrimination  of  two  points  of  a  compass. 

2.  Protopathic  sensibility,  or  the  recognition  of  painful 
cutaneous  stimuli  and  the  extremes  of  heat  and  cold.  This 
form  of  sensation  is  unaccompanied  by  any  definite  appreciation 
of  locality.  The  sensibility  of  the  viscera  is  probably  of 
this  type. 

3.  Deep  sensibility,  consisting  of  the  sense  of  passive 
position  and  of  movement,  and  the  appreciation  of  pressure, 
which  if  excessive  may  produce  pain.  The  fibres  conducting 
deep  sensation  run  with  the  motor  nerves  and  are  distributed 
to  the  muscles,  tendons,  joints  and  periosteum. 

Section  of  a  peripheral  nerve  is  followed  by  loss  of  proto- 
pathic sensibility  over  a  variable  area,  corresponding  approxi- 
mately to  the  distribution  of  the  nerve  on  the  skin.  Epicritic 
sensibility  is  abolished  over  a  somewhat  larger  area. 

In  the  zone  of  epicritic  overlap,  the  sensibility  is  charac- 
terised by  the  great  discomfort  of  a  painful  stimulus,  its 
widespread  diffusion,  and  want  of  localisation.  Deep  sensibility 
is  abolished,  if  the  divided  nerve  contains  both  motor  and 
sensory  fibres. 

In  cases  of  nerve  injury,  not  amounting  to  complete 
division  of  the  nerve,  all  forms  of  sensibility  may  be  affected, 
but  in  slight  lesions  the  epicritic  loss  may  be  the  only  type 
of  impaired  sensibility. 

The  above  facts  also  hold  good  for  the  limb  plexuses  and 
the  posterior  nerve  roots,  with  this  difference— that  the  nearer 
the  lesion  is  to  the  central  nervous  system  the  smaller  becomes 
the  zone  of  overlap  of  epicritic  over  protopathic  loss.  In  root 
lesions  (as  elsewhere  shown,  p.  104)  the  protopathic  loss  may 
even  be  more  extensive  than  the  epicritic.  It  has  therefore 
been  stated  by  Head  as  an  axiom  that  the  posterior  nerve  root 
is  the  unit  of  protopathic,  and  the  peripheral  nerve  the  unit 
of  epicritic  sensibility. 


ANATOMY  AND  PHYSIOLOGY  15 

The  spinal  cord 

In  the  discussion  of  the  conduction  of  sensation  in  the 
spinal  cord,  we  enter  upon  a  subject  of  great  complexity. 

All  varieties  of  sensation  are  conducted  into  the  spinal 
cord  through  one  or  more  of  the  posterior  root  bundles  already 
described.  Some  forms  of  sensation  are  transmitted  at  once 
to  the  opposite  side  of  the  spinal  cord  from  the  entering  pos- 
terior roots,  and  others  are  conducted  upwards  along  the  same 
side.  It  is  well  known  that,  in  consequence  of  injury  or  disease 
of  the  spinal  cord,  one  form  of  sensation  may  be  abolished 
while  another  may  be  retained,  an  observation  which  indicates 
that  different  forms  of  sensation  are  conducted  along  separate 
tracts.  For  example,  it  is  not  uncommon  to  find  that  the 
sensibility  to  painful  stimuli  may  be  lost,  while  tactile  sensi- 
bility is  retained  ;  and  that  if  painful  sensibility  is  abolished, 
the  sensibility  to  heat  and  cold  is  also  in  abeyance. 

The  conduction  of  sensation  may  best  be  studied  by 
observing  the  defects  of  sensibility  following  partial  lesions 
of  the  spinal  cord  in  man. 

Scheme  showing  the  Brown-Sequard  '  Symptom- 
Complex/  based  on  Head's  Observations 

Side  of  lesion.  Side  opposite  lesion. 

Motor  paralysis.  No  paralysis. 

Retention     of     tactile,    light      Tactile    and     light    pressure 
pressure,  painful  and  ther-  sensibilities    may    or    may 

mal  sensibilities.  not  be  impaired. 

Painful  and  thermal  sensibili- 
ties abolished. 
Painful  pressure  retained.  Painful  pressure  abolished. 

Impairment    or    abolition    of     Retention  of  sense  of  position 
tactile  discrimination,  and  and  of  tactile   discrimina- 

sense  of  position  of  limbs.  tion. 

Retention  of  cutaneous  local-     Cutaneous     localisation     de- 
isation.  pends    upon    the    state    of 

tactile  sensibility. 

From  a  study  of  this  Table  it  is  apparent :   (1)  that  some 
forms  of   sensation  (pain  and  temperature  sensibility)   cross 


16  NERVOUS  DISEASES 

soon  after  their  entrance  and  pass  up  the  opposite  side  of  the 
rord  :  (2)  that  other  forms  of  sensation — the  sense  of  passive 
position  and  of  movement  and  tactile  discrimination  (Head)  — 
do  not  cross,  but  pass  up  on  the  same  side  as  the  entering 
posterior  nerve  roots ;  (3)  that  tactile  sensibility  may  or 
may  not  be  abolished  on  the  side  opposite  the  lesion  ;  and 
(i)  that  the  sense  of  cutaneous  localisation  may  be  dissociated 
from  and  remain  intact,  when  the  sense  of  passive  position  is 
absent  (Head). 

In  the  spinal  cord,  the  varieties  of  sensory  loss  are  not 
recognised  in  the  same  combinations  as  those  found  in  lesion 
of  a  peripheral  mixed  nerve.  On  their  entrance  into  the  cord, 
a  rearrangement  of  the  sensory  fibres  of  the  posterior  roots 
takes  place,  so  that  some  forms  of  sensation  cross  and  pass 
up  the  opposite  side,  while  others  remain  upon  the  same  side. 
The  fibres  of  the  posterior  nerve  roots,  which  pass  directly  up 
the  same  side  of  the  cord,  are  the  long  extrinsic  fibres  of  the 
posterior  columns.  These  conduct  the  impressions  subserving 
the  senses  of  passive  position,  of  movement,  and  of  tactile 
discrimination. 

Of  the  other  posterior  root  fibres,  some  were  seen  to  cross 
indirectly  into  the  ventro-lateral  part  of  the  opposite  lateral 
column,  and  to  pass  upwards  as  the  spino-thalamic  system, 
which  tract  is  believed  by  some  authorities  to  conduct  the 
impressions  of  pain  and  temperature  sensibility.  In  opposi- 
tion to  this  view  is  the  fact  that  destruction  of  the  gelatinous 
substance  of  the  posterior  horn  at  the  upper  end  of  the  cord 
is  followed  by,  among  other  phenomena,  abolition  of  contra- 
lateral painful  sensibility — a  fact  which  would  point  to  the 
posterior  horn  of  grey  matter,  or  the  adjacent  tissues  as  con- 
ductors of  painful  sensibility. 

The  tracts  which  conduct  tactile  and  pressure  sensibilities 
are  mainly  crossed,  but  they  do  not  decussate  so  soon  after 
their  entrance  into  the  cord  as  those  for  pain  and  tempera- 
ture ;  hence  these  impressions  are  probably  conducted  for  a 
short  distance  in  the  homo-lateral  posterior  columns,  and, 
higher  up,  in  the  ventro-lateral  tracts  of  the  opposite  side. 

We  have  endeavoured  to  co-ordinate  and  arrange  these 
facts  in  the  subjoined  Table,  which  shows  :  (1)  the  three  main 
forms  of  sensibility  found  in  the  peripheral  nerves ;  (2)  the 
varieties  and  kinds  of  sensation  forming  the  three  primary 


ANATOMY  AND  PIIY8IOLOUY 


17 


groups ;  (3)  their  position  in  the  spinal  cord,  whether  homo- 
or  contra-lateral  to  the  entering  posterior  nerve  roots ;  and 
(4)  the  tracts  in  the  cord  by  which  the  several  forms  of 
sensation  are  probably  conducted. 


Sensory 

Relation  in 

grouping  in 

Variety  of  sensation. 

cord  to 

Position  in  columns  of  cord. 

the  nerves. 

entering  root. 

(1.  Light  touch 

Partly  crossed 

Partly  homo-lateral   pos- 

Partly un- 

terior    column,     partly 

crossed 

antero-lateral  column  of 
opposite  side 

Epicritic 

J  2.  Discrimination 

Uncrossed 

Posterior  column 

sensibility 

3.  Localisation 

As  for  light 
touch 

As  for  light  touch 

4.  Moderate  shades 

Crossed 

(a)  Spino  thalamic  system 

^         of  temperature 

of  antero-lateral  columns 

/  5.  Extremes  of 

Crossed 

or 

Protopathic 

temperature 

sensibility 

[6.  Pain 

(b)  Posterior  grey  horns 

Crossed        , 

7.  Sense  of  passive 

Uncrossed 

Posterior  column 

position 

Deep 

sensibility 

i  8.  Contact  and  pres- 

As for  light 

As  for  light  touch 

sure 

touch 

9.  Painful  pressure 

Crossed 

As  for  pain 

The  mid-  and  hindbrain 

As  already  shown,  two  main  routes  exist  for  sensory 
conduction  in  these  localities : — 

1.  The  mesial  fillet,  which  conducts  the  sensations  con- 
veyed by  the  contra-lateral  posterior  columns  of  the  cord. 
Degeneration  of  this  structure  is  not  followed  by  loss  of 
tactile  and  painful  sensibility. 

2.  The  spino-thalamic  system,  which  conveys  the  impres- 
sions of  touch,  pain,  and  temperature  sensibilities  from  the 
corresponding  tracts  in  the  antero-lateral  columns.  There 
are  cases  on  record  of  hemorrhage  and  softening  of  the 
tegmentum,  and  of  experimental  division  of  this  structure 
in  which  the  fillet  was  intact,  but  which  were  accompanied 
by  anaesthesia  and  analgesia  of  the  opposite  side. 

All    forms   of  sensation    are    gathered    together    in    the 

2 


18  NERVOUS  DISEASES 

ventrolateral  parts  of  the  optic  thalamus,  as  destruction  of  this 
ganglion  is  followed  by  complete  loss  of  all  forms  of  cutaneous 
sensibilitj"  upon  the  opposite  side  of  the  body,  as  well  as  by 
loss  of  the  sense  of  position  and  of  movement  of  the  limbs. 

Cortical  sensory  centres 

Recent  investigation  confirms  the  early  view  that  motion 
and  sensation  have  separate  centres  in  the  cerebral  cortex. 
There  is  accumulating  clinical  evidence  that  the  post-central 
gyrus  is  the  cortical  area  for  the  senses  of  passive  position 
and  of  movement,  for  tactile  localisation  and  discrimination, 
as  well  as  for  cutaneous  sensibility. 

There  is  also  evidence  that  the  post-central  area,  like  the 
precentral,  is  further  subdivided  into  zones  corresponding  to 
the  different  portions  of  the  body.  The  sensory  cortical 
centres  are  situated  immediately  posterior  to  the  correspond- 
ing motor  centres. 

Facts  bearing  upon  the  cortical  centres  for  pain  and  tem- 
perature sensibility  are  less  satisfactory.  Experimental 
evidence  favours  the  gyrus  fornicatus,  but  clinical  observation 
supports  the  view  that  the  sensibilities  of  pain  and  tempera- 
ture have  cortical  centres  in,  or  immediately  behind,  the 
post-central  gyrus. 

Campbell's  observations  have  shown  that  the  post-central 
area  proper  merges  posteriorly  into  an  '  intermediate  post- 
central,' which  extends  into  the  parietal  lobe. 

These  areas  contain  higher  psychical  sensory  centres  of 
complex  character,  in  which  impressions  derived  from  the 
various  forms  of  cutaneous  and  deep  sensibility  are  correlated, 
and  translated  into  definite  conceptions. 

THE  CEREBELLAR   SYSTEM 

Anatomy.  The  cerebellum  consists  of  a  large  mass  of 
cortical  grey  matter  and  several  sub-cortical  ganglionic 
structures:  the  corpus  dentatum,  the  roof  nuclei,  and  probably 
also  the  nucleus  of  Leiters. 

The  nucleus  of  Deiters  occupies  a  prominent  position 
upon  the  dorso-lateral  aspect  of  the  pons  Varolii.  It  is 
characterised  by  the  presence  of  large  multipolar  ganglion 


ANATOMY  AND  PHYSIOLOGY  19 

cells,  similar  to  those  seen  in  the  anterior  horns  of  the  spinal 
cord.  It  has  important  relations  with  the  vestibular  nerve 
and  the  spinal  cord. 

The  cerebellum  is  connected  with  the  brain  stem  by  afferent 
and  efferent  tracts. 

The  afferent  cerebellar  tracts  are  the  following  :— 

1.  The  dorsal  or  direct  spino-cerebellar  tract  of  Flechsig. 
The  fibres  of  this  tract  arise  as  the  axis-cylinder  processes 
of  the  cells  of  Clarke's  group,  which  is  situated  in  the 
intermediate  grey  matter  of  the  spinal  cord,  mainly  in  the 
lower  dorsal  and  upper  lumbar  regions.  Clarke's  group  is 
in  intimate  relation  with  certain  of  the  posterior  root  fibres, 
as  after  section  of  the  posterior  roots,  or  in  association  with 
the  posterior  columnar  degeneration  of  tabes  dorsalis,  the 
medullated  fibres  of  Clarke's  group  atrophy  and  disappear. 
This  group  of  cells  therefore  appears  to  be  an  intermediate 
station  in  the  afferent  spino-cerebellar  system.  Issuing  from 
Clarke's  cell-group,  the  fibres  of  the  dorsal  spino-cerebellar 
tract  take  up  a  position  in  the  postero-lateral  periphery  of 
the  spinal  cord,  and  ascend  to  the  restiform  body.  In  this 
structure  they  are  transmitted  to  the  cerebellum  and  are 
projected  on  to  the  dorsal  surface  of  the  middle  lobe. 

2.  The  ventral  spino-cerebellar  tract,  forming  a  portion 
of  the  ascending  antero-lateral  tract  of  Gowers.  The  fibres 
of  this  tract  arise  as  axis-cylinder  processes  of  cells  situated 
within  the  posterior  horn  of  the  same  and  the  opposite 
side.  These  cells  also  stand  in  relation  to  certain  of 
the  entering  posterior  root  fibres.  In  the  antero-lateral 
periphery  of  the  spinal  cord  the  fibres  of  the  ventral 
spino-cerebellar  tract  lie  external  to  the  descending  antero- 
lateral (vestibular)  fibres,  and  also  to  the  ascending  spino- 
thalamic fibres.  The  tract  passes  upwards  into  the  bulb  and 
pons,  as  high  as  the  root  fibres  of  the  fifth  cranial  nerve. 
Here  it  bends  backwards  into  the  superior  cerebellar  peduncle, 
along  which  it  is  transmitted  to  the  cerebellum,  and  termi- 
nates upon  the  ventral  surface  of  the  middle  lobe. 

3.  The  vestibular  nerve,  as  described  on  p.  61. 

4.  Some  fibres  would  appear  to  pass  from  the  nuclei  of 
the  posterior  columns  upon  the  same  side,  but  the  existence 
of  this  connexion  has  been  denied  by  recent  observers. 

5.  Fibres  coming  from  the   cerebral  cortex  are  supposed 

2  * 


20  NERVOUS  DISEASES 

to  pass  to  the  pontine  nuclei,  and  to  be  thence  transmitted 
by  way  of  the  middle  peduncle  to  the  cerebellum. 

6.  The  olivo-cerebellar  connexion. 

The  efferent  cerebellar  connexions  are  the  following : — 

1.  The  brachium  conjunctivum  emerges  from  the  corpus 
dentatum  cerebelli  and  passes  by  way  of  the  superior  cere- 
bellar peduncle  to  the  tegmentum  cruris,  where  it  decussates 
with  its  fellow  from  the  opposite  side.  It  then  passes  for- 
wards to  terminate  partly  in  the  red  nucleus  and  partly  in 
the  ventral  portions  of  the  optic  thalamus.  There  is  no 
anatomical  evidence  of  a  direct  passage  of  fibres  from  the 
brachium  to  the  cerebral  cortex,  although  it  is  surmised  that 
an  indirect  transference  of  cerebellar  fibres  may  take  place  by 
way  of  the  internal  capsule  and  centrum  ovale. 

2.  The  efferent  fibres  of  the  middle  cerebellar  peduncle 
pass  from  the  corpus  dentatum  of  one  cerebellar  lobe  to  the 
nucleus  pontis  and  tegment  of  the  opposite  side. 

3.  An  important  indirect  cerebellar  efferent  tract  is  that 
which  passes  from  the  large-celled  nucleus  of  Deiters  through 
the  bulb  and  occupies  the  antero-lateral  periphery  of  the 
spinal  cord  throughout  its  whole  extent.  This  tract,  known 
as  the  vestibulospinal,  or  descending  antero-lateral  tract, 
degenerates  only  after  destructive  lesion  of  the  large  cells  of 
Deiters's  nucleus,  and  would  appear  to  be  the  agent  through 
which  the  cerebellum  exerts  a  controlling  influence  upon  the 
trunk  and  limbs. 

Certain  intra-cerebellar  tracts  are  also  present — viz.,  those 
which  connect  the  cerebellar  cortex  with  the  nucleus  dentatus, 
the  roof  nuclei,  and  the  nucleus  of  Deiters. 

It  will  be  seen  from  the  above  description  that  the 
connexions  of  the  cerebellum  are  partly  crossed  and  partly 
uncrossed.  The  crossed  connexions  are  the  superior  and 
middle  cerebellar  peduncles,  the  ventral  spino-cerebellar  tract, 
and  the  olivo-cerebellar  bundle.  The  uncrossed  connexions 
are  the  dorsal  spino-cerebellar  tract,  and  the  vestibulo-spinal 
tract  from  the  nucleus  of  Deiters. 

Physiology.  Considerable  divergence  of  opinion  still  exists 
as  to  the  functions  of  the  cerebellum.  The  effects  of  its  experi- 
mental ablation  (in  whole  or  in  part)  have  been  studied  by  a 
number  of  observers,  and  the  general  effects  of  this  operation 
may  be  briefly  stated. 


ANATOMY  AND  PHYSIOLOGY  21 

After  removal  of  the  whole  cerebellum  profound  dis- 
turbances of  equilibration,  instability,  and  incoordination  of 
movement  of  the  limbs,  trunk,  and  head  are  observed.  These 
immediate  consequences  of  the  lesion  are  followed  by  long- 
continued  unsteadiness  of  the  trunk  and  limbs  on  muscular 
effort,  tremor,  and  nystagmus.  These  symptoms  may  persist 
for  an  indefinite  period,  but  tend  to  diminish  in  course  of 
time. 

After  removal  of  one-half  of  the  cerebellum,  the  persistent 
symptoms  are  similar  in  all  respects  to  those  observed  after 
total  extirpation,  but  are  confined  to  the  side  of  the  lesion. 

The  symptoms  observed  after  division  of  the  cerebellar 
peduncles  are  similar  to  those  following  a  unilateral  ex- 
tirpation, but  there  is  a  greater  tendency  to  roll  round 
the  longitudinal  axis  towards  the  side  of  the  lesion.  There 
is  also  a  more  marked  incurvation  of  the  vertebral  axis 
towards  the  side  of  the  lesion,  and  a  greater  tendency  for 
the  homo-lateral  limbs  to  assume  an  attitude  of  adduction 
and  flexion,  and  the  contra-lateral  limbs  one  of  abduction  and 
extension.  In  these  respects  the  symptoms  are  comparable 
to  those  following  division  of  the  vestibular  nerve  (p.  62). 

Compensation,  or  restitution  of  function,  occurs  to  a  large 
extent  after  severe  destructive  lesions,  but  certain  permanent 
signs  remain  in  the  shape  of  cerebellar  ataxy,  a  reeling  gait, 
and  an  unsteady  and  ungraduated  action  of  the  limbs  on 
voluntary  movements. 

These  permanent  symptoms  are,  in  all  respects,  comparable 
to  what  is  observed  with  the  slower  and  less  complete  lesions 
of  the  cerebellum  in  man.  In  these  cases  are  found  a  reeling, 
unsteady,  or  drunken  gait,  and  an  inco-ordinated  or  ungradu- 
ated action,  more  especially  of  the  movements  of  the  arm  on 
volitional  effort,  although  definite  intention  tremors  are  not 
commonly  seen  in  lesions  limited  to  the  cerebellum. 

Some  degree  of  enfeebled  muscular  tone  (hypotonia),  affecting 
more  especially  the  muscles  of  the  neck  and  back,  but  also 
of  the  limbs  has  been  observed. 

True  motor  paralysis  does  not  occur  in  consequence  of 
lesion  of  the  cerebellum  as  such,  although  some  degree  of 
muscular  asthenia,  or  defect  in  the  force  of  the  muscular 
contraction,  has  been  described  by  some  observers. 

Through  its  superior  peduncle  the  cerebellum  may  exert 


22  NERVOUS  DISEASES 

an  indirect  influence  (tonic  or  trophic)  upon  the  Rolandic  area 
of  the  opposite  side.  The  connexions  of  the  superior  peduncle, 
however,  are  more  directly  with  the  thalamic,  sub-thalamic, 
and  rubral  regions  of  the  midbrain.  Sherrington1  has  shown 
that  division  of  both  superior  peduncles  and  the  tegmentum 
pontis  induces  a  condition  of  '  decerebrate  rigidity,'  not 
unlike  that  observed  after  removal  of  the  entire  cerebellum, 
and  Holmes 2  has  recorded  some  cases  of  tremor  having  the 
features  of  that  observed  after  division  of  the  superior 
cerebellar  peduncle  in  monkeys,  in  which  the  lesion  was  a 
tumour  involving  the  sub-thalamic  and  rubral  regions.  It  is, 
therefore,  not  unlikely  that  the  influence  of  the  cerebellum  is 
mainly  upon  the  basal  structures  just  described,  and  through 
them  by  way  of  the  rubro-spinal  and  vestibulo-spinal  tracts 
upon  the  musculature  of  the  trunk  and  limbs. 

It  has  been  recently  shown  (Horsley  and  Clarke) 3  that 
the  cortex  of  the  cerebellum  is  electrically  inexcitable.  The 
projection  of  the  cerebellar  afferent  tracts  on  to  the  cortex 
cerebelli  favours  the  view  that  the  whole  cortex,  and  more 
especially  that  of  the  middle  lobe,  is  an  afferent  receptive 
centre.  According  to  these  observers  the  intrinsic  and  para- 
cerebellar  nuclei  are  the  cerebellar  efferent  mechanisms. 
Their  action  when  stimulated  is  tonic ;  excitation  of  the 
intrinsic  nuclei  provokes  homo-lateral  movements  of  the  eyes 
and  limbs ;  and  of  the  para-cerebellar  nuclei,  extension  of  the 
lower  limbs  and  hyper-extension  of  the  neck  and  trunk. 

According  to  Sherrington,  the  cerebellum  is  the  '  head 
ganglion  of  the  proprio-ceptive  system,'  of  which  the  labyrinth 
is  the  most  important  peripheral  organ.  Impressions  from 
this  organ  reach  the  cerebellum  by  the  vestibular  nerve,  while 
those  from  the  joints,  tendons,  skin,  and  muscles  are  conducted 
by  the  dorsal  and  ventral  spino-cerebellar  tracts. 

\  Sherrington,  Brain,  1900. 

-  Holmes,  Brain,  1904. 

:!  Horsley  and  Clarke,  Brain,  1905 


Part    II 

THE  EXAMINATION  OF  THE  NERVOUS 
SYSTEM  IN  A  CASE   OF   NERVOUS   DISEASE 

For  the  proper  investigation  of  a  case  of  nervous  disease 
a  systematic  and  thorough  method  of  examination  should 
be  adopted.  The  use  of  a  routine  plan  is  desirable,  and 
will  save  the  physician  from  mistakes  in  the  diagnosis  of  a 
class  of  disease  in  which  the  symptoms  and  signs  present 
many  variations.  The  discrimination  of  important  from 
unimportant  signs  is  acquired  by  knowledge,  and  the  value 
of  negative  in  contrast  to  positive  facts  can  only  be 
appreciated  by  experience.  Such  experience  enables  the 
physician  to  seize  upon  the  essential  points  of  each  case,  and 
to  extend  his  examination  without  neglecting  the  subordi- 
nate features.  It  should  always  be  kept  in  mind  that  the 
least  obvious  may  be  the  most  important  sign. 

GENERAL  METHOD  AND  SCHEME  OF  EXAMINATION 

Before  commencing  the  examination  of  a  case  of  nervous 
disease,  inquiry  should  be  directed  to  the  following : — ■ 
i.  Complaint. 

2.  Duration. 

3.  Family  history.  The  family  history  of  a  patient 
suffering  from  nervous  disorder  is  important.  In  the  case  of 
functional  disorders  inquiry  should  be  made  particularly  into 
family  epilepsy,  insanity,  alcoholism,  and  the  minor  psychoses, 
such  as  neurasthenia,  psychasthenia,  hysteria,  morbid  fears, 
obsessions,  and  tics.  In  organic  nervous  diseases  inquiry 
should  be  directed  towards  such  diathetic  states  as  syphilis, 
gout,  rheumatoid  arthritis,  rheumatism,  tuberculosis,  and 
vascular  degeneration. 

23 


24  NERVOUS  DISEASES 

4.  Personal  history.  The  history  of  the  patient's  health 
prior  to  the  onset  of  the  illness  may  throw  light  upon  the 
malady.-  This  should  be  investigated  in  a  chronological  order 
commencing  with  birth,  the  age  at  the  onset  of  teething,  and 
whether  it  was  accompanied  by  convulsions,  the  age  when 
the  patient  walked  and  talked,  the  diseases  of  infancy,  the 
incidence  of  maladies  such  as  bed-wetting,  night  terrors, 
somnambulism,  epilepsy,  chorea,  middle  ear  disease,  and 
tuberculosis.  Of  illnesses  during  later  years,  the  more  im- 
portant are  influenza,  the  exanthemata,  diphtheria,  enteric 
fever,  and  acute  rheumatism.  Venereal  infection,  more 
especially  syphilis,  should  be  a  subject  of  careful  inves- 
tigation ;  in  women,  information  on  this  point  can  only 
be  obtained  by  collateral  evidence,  such  as  relates  to 
miscarriages.  The  habits  of  the  patient  regarding  alcohol, 
tobacco,  exercise,  and  diet  should  also  be  the  subject  of 
investigation. 

The  occupation  should  be  considered  with  special  refer- 
ence to  toxic  trades,  such  as  working  in  lead,  phosphorus, 
mercury,  and  arsenic ;  to  those  which  favour  the  de- 
velopment of  the  neuroses — such  as  that  of  writer, 
pianist,  tailor,  or  seamstress ;  and  to  professions  requir- 
ing sedentary  habits  and  mental  strain  and  stress.  The 
personal  habits  and  circumstances  of  the  patient  also  require 
investigation. 

5.  The  illness.  The  time,  the  occasion,  and  the  mode 
of  onset  of  the  first  symptoms  are  especially  important.  The 
mode  of  onset  is  valuable  as  an  indication  of  the  nature  of 
the  lesion,  and  may  be  the  point  upon  which  the  diagnosis 
turns.  Inquiry  may  reveal  the  existence  of  previous  tran- 
sitory symptoms,  which  were  regarded  as  of  little  or  no 
significance.  The  course  of  the  malady  may  be  progres- 
sive, regressive,  or  intermittent ;  or  the  symptoms  may  be 
stationary.  The  presence  or  absence  of  headache,  vertigo, 
vomiting,  fits  or  faints  should  be  noted. 

Assigned  causes  given  by  the  patient  or  his  friends  require 
scrutiny  at  the  hands  of  the  physician,  as  they  have  often  to 
be  rejected.  The  physical  and  mental  condition  should  now 
be  examined. 

(1)  The  plujsical  condition.  The  general  physical  develop- 
ment, the  state  of  nutrition  and  complexion  of  the   patient 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        25 

should  be  observed,  and  notice  taken  of  any  morbid  ap- 
pearances— such  as  syphilitic  teeth,  scars  and  cicatrices, 
keratitis,  and  '  stigmata  of  degeneration.'  These  are  seen 
more  especially  in  high,  narrow,  or  deformed  palates,  in 
abnormal  size  or  shape  of  the  ears,  in  cranio-facial  asymmetry, 
in  displaced,  malformed  or  badly  enamelled  teeth,  in  dispro- 
portionate size  of  the  limbs,  hare-lip,  in  anomalies  of  the 
fingers,  skin,  hair,  and  nails,  strabismus  and  marked  errors 
of  refraction — such  as  high  myopia  and  hypermetropia. 

An  examination  of  the  skull  as  to  its  size,  shape,  and  con- 
figuration should  never  be  omitted.  Notice  should  also  be 
taken  of  scars,  nodes,  or  any  other  abnormalities  which  may 
be  present. 

(2)  The  mental  and  emotional  condition.  The  memory  both 
for  recent  and  past  events,  the  power  of  concentration  and 
attention,  and  the  state  of  intelligence  will  be  revealed  during 
the  examination  into  the  history  of  the  illness  by  the 
replies  which  are  given  to  questions.  The  physician  will  also 
observe  such  symptoms  as  depression,  exaltation,  irritability, 
excitement,  loss  of  self-control,  the  presence  of  delusions  or 
hallucinations,  stupor,  delirium,  and  coma. 

In  many  cases,  evidence  of  the  slighter  degrees  of  mental 
change  may  only  be  obtained  by  information  derived  from  the 
friends  or  relatives. 


Examination  op  the  Special  Senses  and  the  Ceanial  Neeves 

Smell 

In  all  cases  presenting  alterations  of  the  sense  of  smell,  a 
local  or  rhinoscopic  examination  should  be  made.  If  the  nose 
is  free  from  local  disease,  the  sense  of  smell  may  be  tested  by 
the  inhalation  of  odorous  substances— such  as  peppermint, 
asafcetida,  oil  of  cloves,  camphor  or  musk ;  each  nostril 
being  examined  separately.  The  patient  is  tested  to  ascer- 
tain whether  he  smells.  If  the  sense  of  smell  and  the 
ability  to  distinguish  odours  are  present,  the  nerves  are 
intact.  Ammonia  or  other  substances  likely  to  irritate  the 
branches  of  the  fifth  nerve  within  the  nose  should  not  be 
used. 


26  NERVOUS  DISEASES 


Taste 

The  most  accurate  method  of  testing  the  sense  of  taste 
lies  in  stimulating  the  sensory  taste-fibres  by  a  weak  galvanic 
current,  applied  by  twisting  two  insulated  copper  wires  to- 
gether, the  ends  of  which  are  exposed  and  separated  one  or 
two  mm.  apart. 

For  ordinary  purposes  the  anterior  two-thirds  may  be 
tested  in  the  following  manner.  The  patient  is  told 
to  protrude  the  tongue,  which  is  then  gently  cleaned. 
Substances  in  the  form  of  white  powders — such  as  quinine, 
salt  or  sugar,  are  then  rubbed  on  to  its  surface,  each  side 
being  separately  tested,  and  the  patient  told  to  indicate  by 
raising  his  finger,  as  soon  as  he  appreciates  a  sensation  of 
taste,  and  then  to  write  what  he  tastes,  the  tongue  not  being 
retracted  into  the  mouth. 

To  test  the  posterior  third  it  is  necessary  to  drop  strong 
solutions  of  these  substances  on  to  the  back  of  the  tongue. 

Vision 

The  following  should  be  tested  and  examined  :  — 

(a)  The  acuteness  of  vision  ; 

(b)  The  fields  of  vision  ; 

(c)  The  optic  discs. 

As  impairment  of  visual  acuteness  and  of  the  fields  of 
vision  may  be  due  to  errors  of  refraction — myopia,  hyper- 
metropia,  and  astigmatism,  or  to  local  diseases  of  the  media 
or  retina — it  is  important  for  the  examiner  to  exclude  all 
such  factors  before  assigning  any  visual  defect  to  nervous 
causes. 

(a)  The  visual  acuteness  for  distant  objects  is  tested  by 
means  of  Snellen's  test-types,  which  are  constructed  so  that 
a  person  placed  six  metres  away — at  which  distance  the 
rays  of  light  are  practically  parallel — may  have  his  vision 
expressed  in  fractions.  The  numerator  records  the  distance 
of  the  patient  from  the  type,  the  denominator  the  distance 
at  which  each  letter  should  be  read  by  the  normal  eye.  The 
largest  letters  should  be  read  under  normal  conditions  at 
sixty  metres ;  therefore,  if  a  patient  can  only  read  the  largest 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        27 

letters  at  six  metres,  the  formula  is  V  =  -(fo.  If,  on  the 
other  hand,  the  patient  can  read  the  small  typo  at  six  metres, 
V  =  |.  The  test-types  should  be  placed  in  a  good  light,  and 
each  eye  tested  separately.  If  the  patient  is  unable  to 
read  any  of  the  letters  at  six  metres,  he  should  be  tested 
to  see  at  what  distance  he  can  correctly  count  fingers,  and  the 
result  recorded  thus  :  V=  fingers  at  (so  many)  feet.  If  he  is 
unable  to  see  fingers  or  hand  movements,  his  perception  of  light 
is  tested  either  by  alternate  shading  and  exposure  of  the  eye  to 
light,  or,  better,  by  throwing  a  ray  from  an  ophthalmoscopic 
mirror  upon  the  eye  in  a  dark  room. 

For  testing  the  acuteness  of  near  vision,  Jaeger's  test-types 
are  used.  The  accommodative  power  of  a  person  reading  the 
smallest  type  at  the  normal  distance  of  from  six  to  eight 
inches  being  recorded  by  the  formula  :  Jaeger  1  at  6-8 
inches. 

Where  impairment  of  acuteness  is  due  to  hypermetropia, 
myopia,  or  astigmatism,  vision  is  recorded  in  the  following 
formula :  V  =  ^,  (with  stated  correction)  V  =  |  or  £  (as  the 
case  may  be). 

(b)  The  fields  of  vision.  The  only  accurate  and  scientific 
method  of  testing  the  visual  fields  is  by  means  of  the  peri- 
meter, and  recording  upon  charts  the  result  of  the  investiga- 
tion. This  method,  when  properly  carried  out,  will  reveal 
not  only  the  existence  of  concentric  limitations  of  the  fields 
and  hemianopsia,  but  also  localised  areas  of  defective  vision 
(scotomata).  It  is  advisable  to  test  not  only  the  field  for 
white,  but  also  for  colours.  Normally,  the  field  for  white  is 
the  most  extensive,  and  within  this  area  are  the  fields  for 
blue,  red,  and  green  respectively. 

For  practical  purposes,  a  general  idea  of  the  visual  fields 
may  be  obtained  by  the  following  test.  The  patient  is  placed 
with  his  back  to  the  light,  each  eye  being  examined  separately. 
The  examiner  sits  about  two  feet  from  the  patient  and  directs 
him  to  look  steadily  into  whichever  of  the  observer's  eyes  is 
opposite  the  one  to  be  examined.  Thus,  when  the  left  eye  is 
being  examined  he  will  fix  the  examiner's  right  eye.  The 
examiner  then  brings  his  finger  towards  the  centre  from 
various  parts  of  the  periphery,  and,  by  asking  the  patient  to 
state  as  soon  as  he  sees  the  finger,  he  obtains  an  approximate 
idea  of  the  size  of  the  visual  fields. 


28  NERVOUS  DISEASES 

If  the  state  of  the  patient  renders  such  an  examination 
impossible,  the  fields  may  be  tested  by  quickly  bringing  the 
hand  in  from  various  parts  of  the  periphery  towards  the  eye, 
and  a  note  made  as  to  whether  blinking  occurs  when  the 
eye  is  threatened.  If  hemianopsia  is  present,  no  response 
is  obtained  when  the  finger  is  brought  in  from  the  blind 
field. 

The  various  defects  of  the  visual  field  are  : — 

1.  Concentric  contraction,  or  diminution  of  the  peripheral 
field. 

2.  Hemianopsia,  or  loss  of  vision  in  one-half  of  the  visual 
field.  This  may  be  horizontal  or  vertical.  The  horizontal 
forms  are  (a)  homonymous  hemianopsia,  or  blindness  of 
the  corresponding  lateral  halves  of  the  visual  fields ;  {b) 
heteronymous  hemianopsia,  or  blindness  of  the  opposite 
lateral  fields  (binasal  or  bitemporal). 

The  vertical  forms  are  superior,  or  blindness  of  the  upper 
half,  and  inferior,  or  blindness  of  the  lower  half  of  the  fields. 

3.  Quadrantic  hemianopsia,  or  blindness  of  the  correspond- 
ing sectors  of  the  visual  fields. 

4.  Scotoma,  central  when  the  macular  region  is  blind, 
annular  when  round  the  fixation  point,  paracentral  when  to 
one  side,  and  eccentric  when  in  the  periphery. 

(c)  The  state  of  the  optic  discs.  This  is  investigated  by 
the  ophthalmoscope.  The  chief  points  to  be  observed  are  the 
colour,  the  presence  or  absence  of  swelling,  the  state  of  the 
margin,  and  the  condition  of  the  central  cup.  In  primary 
atrophy,  the  disc  is  pale,  or  a  parchment  white  or  grey  white 
appearance,  the  edges  are  well  defined,  and  the  lamina  crib- 
rosa  is  clearly  seen  at  the  bottom  of  the  central  cup.  Normally, 
the  outer  half  of  the  disc  is  paler  than  the  inner,  and  the 
diagnosis  of  atrophy  should  be  based  not  upon  pallor  alone, 
but  in  conjunction  with  visual  defect. 

In  secondary  optic  atrophy,  the  disc  is  pale,  the  cup  is 
filled  up,  the  margins  blurred,  and  the  arteries  small  and 
enveloped  by  connective  tissue. 

In  optic  neuritis  the  disc  is  swollen  and  hyperaemic,  the 
margins  blurred,  the  veins  engorged,  and  the  arteries  and 
veins  may  be  buried  in  exudation.  New  vessels  may  be  seen 
on  the  surface  of  the  exudation  and  hemorrhages  may  be 
scattered  about. 


EXAMINATION  OF  THR  NEEVOUS  SYSTEM        29 

In  all  cases  the  state  of  the  fundus  oculi  with  reference  to 
the  retinal  vessels,  the  yellow  spot,  and  the  choroid  should  be 
examined. 

Hearing 

In  all  cases  of  deafness  an  otoscopic  examination  should 
he  made,  for  details  of  which  the  reader  is  referred  to 
special  works  upon  the  diseases  of  the  ear. 

In  neurological  practice  it  is  important  to  test  the  hearing 
in  all  cases  presenting  symptoms  of  deafness,  tinnitus,  or 
vertigo,  more  especially  with  a  view  to  determine  whether 
deafness  arises  from  disease  of  the  sound-conducting  or 
of  the  sound-perceiving  mechanism  of  the  eai\ 

Auditory  acuteness  is  tested  by  several  methods  ;  reliance 
should  not  be  placed  on  any  single  test,  but  on  a  com- 
bination of  several,  of  which  the  following  are  the  most 
important : — 

1.  The  voice.  Although  the  raised,  the  conversational, 
and  the  whispered  voice  may  all  be  used,  the  last  is  the  most 
important.  The  room  should  be  quiet  and  the  patient  placed 
as  far  away  from  the  wall  as  possible.  The  ear  which  is 
not  being  examined  should  be  closed.  The  whispered 
voice  ought  to  be  heard  in  a  quiet  room  at  from  seven  to 
ten  yards,  but  the  test  should  be  carried  out  at  varying 
distances. 

Acuteness  of  hearing  may  be  measured  and  expressed  by 
a  formula,  in  which  the  numerator  denotes  the  distance 
at  which  the  voice  is  heard  by  the  patient,  and  the 
denominator  the  distance  at  which  it  would  be  heard  by  a 
normal  ear. 

2.  The  watch.  This  is  not  a  satisfactory  mode  of  testing 
hearing,  as  it  only  gives  the  auditory  acuteness  for  feeble 
sounds.  The  distance  at  which  a  particular  watch  is  normally 
heard  should  be  known.  When  used,  it  is  preferably  brought 
in  from  a  distance,  the  patient  being  asked  to  indicate  when 
he  hears  it. 

3.  The  tuning-fork.  Forks  of  low  and  high  pitch  should  be 
used.  The  note  produced  by  a  vibrating  tuning-fork  is  heard 
both  through  the  air  and  the  bones  of  the  skull.  In  normal 
states  of  the  ear,  air  conduction  is  better  than  bone 
conduction. 


30  NERVOUS  DISEASES 

In  disorders  of  the  sound-conducting  apparatus — '  obstruc- 
tive deafness ' — the  tuning-fork  is  heard  better  through  the 
bone  than  through  the  air  (BC  ^>  AC).  Conversely,  in  affec- 
tions of  the  sound-perceiving  apparatus — '  nerve  deafness ' — 
the  normal  reaction,  in  which  the  fork  is  heard  through  the 
air  better  than  through  the  bone,  is  present  (AC  >  BC).  These 
reactions  may  be  investigated  by  Weber's  and  Rhine's 
tests. 

Weber's  test.  If  in  a  unilateral  ear  affection  the  stem  of  a 
vibrating  tuning-fork  be  placed  mesially  upon  the  skull,  and 
the  note  heard  in  the  deaf  ear,  the  sound-conducting  apparatus 
is  probably  at  fault  ('middle  ear  deafness').  If  referred  to  the 
normal  ear,  the  lesion  is  in  the  sound-perceiving  apparatus 
( '  internal  ear  deafness  ' ).  If  in  a  bilateral  ear  affection  the 
tuning-fork,  placed  mesially,  is  referred  to  the  less  deaf  ear,  it 
is  evidence  in  favour  of  an  internal  ear  lesion  in  the  more 
deaf  ear. 

mane's  test.  Normally  the  vibrating  tuning-fork  is  heard 
when  held  in  front  of  the  external  auditory  meatus,  after  it 
has  ceased  to  be  heard  upon  the  mastoid  process.  This  is  the 
positive  Rinne  response.  Rhine  +  in  a  deaf  ear  is  evidence 
of  an  affection  of  the  sound-perceiving  apparatus  ('internal 
ear  deafness  ').  If  in  a  deaf  ear  the  fork  is  no  longer  heard 
by  air  conduction,  after  it  has  ceased  to  be  heard  per  osseum 
(Rinne  — ),  it  is  evidence  of  an  affection  of  the  sound-con- 
ducting apparatus  ('middle  ear  deafness'). 

Schwabach's  test.  Here  the  observer  compares  his  own 
bone  conduction,  which  must  be  normal,  with  that  of  the 
patient.  "When  the  vibrating  tuning-fork  placed  on  the 
mastoid  process  of  the  patient  has  ceased  to  be  heard  by  him, 
but  is  still  heard  when  placed  upon  the  skull  of  the  observer, 
a.n  affection  of  the  sound-perceiving  apparatus  is  suspected — 
bone  conduction  is  shortened.  On  the  other  hand,  if  the 
patient  hears  the  fork  after  the  observer  has  ceased  to  do 
so,  an  affection  of  the  sound-conducting  apparatus  is 
suggested — bone  conduction  is  lengthened. 

In  differentiating  between  affections  of  the  sound-conduct- 
ing and  sound-perceiving  apparatus,  it  is  of  value  to  make 
use  of  tuning-forks  of  high  and  low  pitch.  In  lesions  of  the 
former,  the  low  tones  are  lost  first ;  in  lesions  of  the  latter, 
the  perception  of  high  tones  is  lost. 


EXAMINATION  OF   THE   NERVOUS   SYSTEM 


;i 


The  preceding  observations  and  their  clinical  significance 
have  been  summarised  in  the  following  Table  : — 


Table   (modified   from    Chavasse  and  Joubeut)    showing    thk    Chiei 

Phenomena  observed  on  testing  the  Sense  or  Hearing 


Tests. 

Affections  of 
middle  ear. 

Affections  of 
labyrinth. 

Combined    con- 
duction and  per- 
ception affections. 

In  old  people. 

Whispered 
voice 

Heard  badly 

Heard  badly 

Variable 

Variable 

Watch  by  air 
Watch  by  bone 

Fair  percep- 
tion 

Good  percep- 
tion 

Heard  badly 
Not  heard 

Variable 
Variable 

Fair  percep- 
tion 

Not  heard 

m     •        "j  Air 
Tuning-  / 

fork     \  -n 

j  Bone 

Diminished 

Normal 

High  tones 

lost 
Duration 

diminished 

Duration 

diminished 
Diminished 
or  lost 

Well  heard 
Not  heard 

Weber 's  test 

BC^>  AC 

ac  y  BC 

Variable 

AC  >  BC 

Rinne's  test 

Negative 

Positive 

Variable 

Positive 

The  Vestibular  Apparatus 

The  following  tests  may  be  applied  :  — 

1.  Bombergis?n.  The  patient  is  made  to  stand  with  the 
eyes  closed,  first  on  both  feet  placed  together  and  then  on 
each  foot  separately,  and  his  ability  or  inability  to  stand  is 
noted.  In  a  unilateral  ear  lesion,  he  may  tend  to  sway  or 
fall  towards  the  affected  side,  but  his  swaying  movements 
may  be  of  an  indefinite  character. 

2.  Gait  in  walking.  The  patient  is  made  to  '  walk  the 
line  '  with  the  eyes  open  and  closed,  and  his  tendency  to 
sway  or  deviate  is  observed.  He  may  deviate  towards  the 
affected  side.  There  may  be  a  tendency  to  broaden  the  base 
of  support  or  even  to  assume  a  degree  of  ataxic  gait. 

3.  Execution  of  movements  requiring  co-ordinate  control — e.o\ 


32  NERVOUS  DISEASES 

hopping  forwards  and  backwards  with  the  eyes  open  and 
closed.  There  is  an  inability  to  carry  out  such  movements 
without  swaying  or  loss  of  co-ordination. 

4.  Eotation  tests.  The  patient  is  seated  upon  an  easily 
rotating  chair,  which  is  made  to  revolve  at  the  rate  of  ten 
complete  revolutions  in  twenty  seconds.  Rotation  is  stopped 
abruptly  and  the  patient  is  directed  to  turn  the  eyes  in 
extreme  deviation — first  towards  the  side  from  which  rotation 
has  been  made,  and  then  in  the  direction  of  the  rotation. 
The  vestibular  organs  in  both  ears  are  simultaneously  tested, 
but  the  nystagmus  produced  after  stopping  rotation,  tests 
more  definitely  the  apparatus  on  the  side  from  which  the 
rotation  has  been  made.  After  rotation,  there  is  horizontal 
nystagmus  towards  the  side  from  which  rotation  has  been 
made  when  the  eyes  are  deviated  to  that  side.  There  is  an 
absence  of  nystagmus  when  the  eyes  are  deviated  in  the 
direction  of  rotation.  The  duration  of  the  nystagmus,  when 
the  vestibular  organs  are  normal,  is  twenty-five  seconds  or 
more. 

If  the  right  labyrinth  be  injured  or  destroyed,  and  the 
patient  be  rotated  from  left  to  right,  the  nystagmus  produced 
after  stopping  rotation  being  mainly  due  to  the  left  labyrinth 
will  continue  for  twenty-five  seconds,  while  that  produced 
after  rotating  from  right  to  left  may  only  continue  for  ten  or 
fifteen  seconds  or  less. 

5.  Caloric  tests.  When  the  normal  ear  is  syringed  with 
water  below  the  body  temperature,  65°-80°F.,  the  head  being 
held  erect,  there  is  horizontal  and  rotatory  nystagmus  on 
fixing  the  eyes  to  the  opposite  side.  If  water  be  used  above 
the  body  temperature,  110°-118°F.,  horizontal  and  rotatory 
nystagmus  occurs  on  fixing  the  eyes  to  the  same  side.  Here 
each  vestibular  apparatus  is  tested  separately.  If  it  is  de- 
stroyed, or  the  vestibular  nerve  paralysed,  there  is  as  a  rule 
no  nystagmus  on  syringing. 

6.  Galvanic  tests.  By  holding  one  electrode  in  the  hand 
and  placing  the  other  upon  the  mastoid  process,  currents  of 
10-25  milliamperes  may  be  used.  When  the  kathode  is 
applied  to  the  normal  ear,  rotatory  nystagmus  is  produced  on 
fixing  the  eyes  to  the  same  side.  When  the  anode  is 
applied,  there  is  rotatory  nystagmus  on  fixing  the  eyes  to 
the  opposite  side. 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        33 

III.,  IV.,  VI. — Third,  fourth,  and  sixth  nerves 

In  testing  ocular  movements  the  eyes  should  be  examined 
separately  and  together.  The  patient  is  told  to  fix  his  eyes 
upon  the  examiner's  finger,  which  is  held  at  about  three  feet 
away  and  moved  in  upward,  downward,  and  lateral  directions. 
Convergence  is  tested  by  approximating  the  finger  towards 
the  eyes.  Impairment  of  the  ocular  movements  and  any 
abnormal  movement — such  as  nystagmus — will  thus  be 
discerned  ;  the  character  and  direction  of  such  movements 
should  also  be  observed. 

Paralysis  of  an  individual  ocular  muscle  may  show 
itself  by  strabismus — when  the  eye  is  at  rest — and  by 
deficient  movement  in  the  direction  of  its  normal  action. 
In  minor  degrees  of  paralysis  these  signs  may  not  be 
evident  to  the  observer,  although  the  patient  may  complain 
of  double  vision  in  a  specified  direction.  If  this  is  the 
case,  it  is  necessary  to  apply  the  candle  test  in  order  to 
ascertain  the  relation  and  position  of  the  false  image.  In 
this  test,  which  is  carried  out  in  a  dark  room,  one  of  the  eyes 
is  covered  by  a  coloured  glass,  and  the  head  is  fixed  to  prevent 
movement.  A  candle  held  about  two  or  three  yards  away 
from  the  patient  is  moved  in  the  plane  of  action  of  each 
muscle,  and  the  patient  is  told  to  indicate  the  position  of  the 
coloured  and  white  flames,  the  relative  position  of  the  two 
images,  their  relation  to  and  separation  from  each  other. 
The  false  image  is  seen  by  the  paralysed  eye  and  is  displaced 
in  the  direction  of  traction  of  the  paralysed  muscle. 

The  position  of  the  images,  their  relation  to  each  other, 
and  the  symptoms  of  paralysis  of  individual  muscles  are 
described  on  pp.  73  to  75. 

The  pupils.  Normally,  the  pupil  is  round  or  slightly 
oval,  with  a  regular  outline,  and  placed  centrally  in  the  iris. 
The  two  pupils  are  of  equal  size,  and  measure  from  two  to 
four  mm.  in  diameter;  they  are  larger  in  myopic  and  smaller 
in  hypermetropic  persons. 

Irregularity  of  outline  may  result  from  local  conditions — 
such  as  synechiae  or  from  a  defect  of  innervation. 

Eccentricity  of  the  pupil  may  be  congenital,  or  may  result 
from  central  nervous  lesions  ensuing  in  later  life. 

When   examining   the   size  of   the  pupils,  the  amount  of 


34  NERVOUS  DISEASES 

light  falling  on  each  eye  should  be  the  same.  Inequality  of 
the  pupils  may  be  due  to  refractive  errors,  to  local  inflam- 
matory changes,  to  paralysis  or  stimulation  of  the  cervical 
sympathetic  nerve  on  one  side,  to  paralysis  of  the  third 
cranial  nerve,  or  to  unilateral  optic  nerve  atrophy. 

Myosis,  or  small  pupil,  may  be  unilateral  or  bilateral.  In 
myosis,  from  paralysis  of  the  sympathetic,  slight  ptosis  is 
present,  the  reflexes  to  light  and  accommodation  are  pre- 
served, but  lost  to  painful  stimulation ;  cocaine  produces  no 
dilatation,  but  atropine  causes  a  permanent  mydriasis. 

Mydriasis,  or  dilated  pupil,  may  also  be  uni-  or  bilateral. 
If  due  to  paralysis  of  the  third  nerve,  all  the  pupillary  reflexes 
are  lost ;  if  on  the  other  hand  it  be  due  to  stimulation  of  the 
cervical  sympathetic  nerve,  the  reflexes  are  preserved  and 
atropine  causes  an  increase  of  the  dilatation. 

Pupillary  reactions.  (a)  Reaction  to  convergence  and 
accommodation  is  tested  by  making  the  patient  look  from  a 
distant  to  a  near  object  when  a  pupillary  contraction  occurs. 

(b)  Reaction  to  light.  In  testing  the  light-reaction  the 
patient  should  be  placed  so  as  to  face  the  window,  each  eye 
being  tested  separately,  the  one  not  under  observation  being 
covered  by  the  examiner's  hand.  Both  the  direct  and  the 
consensual  reactions  are  tested.  A  pupil  inactive  to  light 
may  respond  on  convergence  or  with  accommodation  (Argyll- 
Robertson  phenomenon).  The  pupil  dilates  normally  on 
stimulation  of  the  skin  of  the  neck  (cilio-spinal  reflex). 

In  cases  of  hemianopsia  the  reaction  of  the  pupil  should 
be  examined  by  throwing  light  respectively  upon  the  seeing 
and  the  blind  halves  of  the  visual  field.  To  do  this  satis- 
factorily a  fine  pencil  of  light  is  required.  If  the  pupil  fails  to 
respond  only  when  light  is  thrown  upon  the  blind  half  of  the 
retina,  it  signifies  that  the  lesion  is  situated  in  the  chiasma  or 
optic  tract.     This  is  the  hemiopic  pupillary  reaction. 

V. — Fifth  or  trigeminal  nerve 

Motor.  The  voluntary  action  of  the  temporal  and  masseter 
muscles  is  tested  by  asking  the  patient  to  clench  the  teeth,  and 
the  action  of  the  external  pterygoid  muscles  by  asking  the 
patient  to  open  his  mouth  against  resistance.  In  cases  of 
unilateral  palsy  the  jaw  deviates  to  the  paralysed   side  and 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        35 

the  patient  is  unable  to  move  the  jaw  to  the  non-paralysed 
side.  An  inability  to  depress  the  lower  jaw  signifies  paralysis 
of  the  internal  pterygoid  muscles.  Degenerative  changes  in 
the  muscles  are  ascertained  by  the  presence  of  atrophy  and 
altered  reactions  to  faradic  and  galvanic  excitation. 

Sensory.  Sensation  is  tested  as  detailed  later  under  the 
examination  of  the  sensory  phenomena  elsewhere  in  the 
body  (p.  39).  It  is  also  necessary  to  test  the  sensibility  of 
the  cornea  and  the  mucous  membranes  of  the  mouth,  nose, 
palate,  and  tongue. 

VII. — Seventh  or  facial  nerve 

The  face  should  be  examined  first  in  repose,  and  any 
asymmetry  noted.  Slight  asymmetry  is  not  important. 
In  paralysis  the  face  may  be  drawn  to  one  side,  either 
by  over-action  of  non-paralysed  muscles,  or  by  contracture 
of  paralysed  muscles.  The  voluntary  movements  should 
next  be  tested  by  such  actions  as  elevation  of  the 
eyebrows,  frowning,  closure  of  the  eyelids,  showing  the  teeth, 
whistling,  and  pouting.  If  paralysis  is  present,  these  move- 
ments will  be  impaired.  The  emotional  movements — such  as 
occur  in  smiling,  laughing,  and  crying — should  be  observed. 
Differences  between  the  degree  of  affection  of  the  upper 
and  lower  parts  of  the  face  are  also  to  be  noted. 

The  electrical  reactions  of  the  muscles,  both  to  faradic 
and  galvanic  stimulation,  should  be  tested  in  all  cases  of 
facial  paralysis  (p.  51). 

IX.,  X.— Ninth  and  tenth  nerves.     Vago-glossopharyngeal 

nerve 

The  points  to  be  investigated  in  connexion  with  this  nerve 
are  the  movements  of  the  pharyngeal  muscles,  the  soft  palate, 
and  the  vocal  cords  ;  and  in  addition  the  action  of  the 
pulmonary,  cardiac,  and  gastric  centres. 

1.  Pharynx  and  soft  palate.  The  signs  of  paralysis 
are — (1)  inability  to  swallow,  (2)  regurgitation  of  fluids 
through,  the  nose,  and  (3)  an  absence  of  movement  of 
elevation  of  the  soft  palate  when  the  patient  is  asked  to 
open  his  mouth  and  say,  '  Ah.' 

In  unilateral  lesions  little  impairment  of  swallowing  occurs, 

3  * 


36  NERVOUS  DISEASES 

but  the  palate  will  be  observed  to  be  drawn  upwards  to  the 
non-paralysed  side  on  phonation. 

The  sensory  functions  are  tested  (a)  as  to  taste  on  the 
posterior  third  of  the  tongue,  and  (b)  as  to  common  sensation 
over  the  soft  palate,  pharynx,  and  fauces.  The  palato- 
pharyngeal reflex  is  tested  by  tickling  the  fauces. 

2.  The  larynx.  Any  changes  in  the  voice  should  be 
noted,  also  the  ability  to  cough  and  phonate,  and  the  presence 
of  stridor.  In  all  cases  presenting  such  symptoms,  a  laryngo- 
scopy examination  ought  to  be  made.  The  symptoms  of  the 
several  forms  of  laryngeal  paralysis  and  the  corresponding 
laryngoscopic  appearances  are  detailed  on  p.  92. 

3.  Alterations  in  the  pulmonary,  cardiac,  and  gastric 
functions — such  as  slow  or  altered  respiration,  rapid  or  slow 
heart's  action,  vomiting  and  hiccough — can  only  be  referred 
to  nerve  disturbances  in  the  absence  of  organic  disease  or 
other  obvious  cause. 

XII. — Twelfth  nerve.     Hypoglossal  nerve 

This  is  tested  by  asking  the  patient  to  protrude  the 
tongue  and  to  put  it  into  his  cheeks.  Loss  of  these  move- 
ments may  be  unaccompanied  by  wasting  of  the  tongue,  when 
due  to  a  lesion  above  the  nucleus.  The  orbicularis  oris  may 
be  affected  in  association  with  nuclear  palsy  of  the  tongue. 
If  the  paralysis  is  unilateral,  the  tongue  on  protrusion  deviates 
to  the  paralysed  side.  In  nerve  lesions  proper  the  tongue  is 
paralysed  on  one  or  both  sides,  and  the  paralysis  is  accom- 
panied by  wasting  and  furrowing  of  its  surface.  The 
commencement  of  the  act  of  swallowing  is  impaired,  and 
articulation  is  defective. 

Examination  op  the  Motoe  System 

The  examination  of  the  motor  system  of  the  trunk  and 
limbs  is  carried  out  to  ascertain  : — - 

1.  The  general  condition  of  the  muscles.  Observation 
should  be  made  upon  the  physique  of  the  patient,  his 
muscular  development  in  relation  to  his  occupation  and 
pursuits,  the  presence  of  deformities,  and  the  attitude  and 
position  of  the  limbs  at  rest  and  on  movement. 

2.  The  presence  or  absence  of  tremor,  spasm,  and  of 
convulsive  or  other  abnormal  movements.     The  character  of 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        37 

such  movements  should  be  observed,  their  range,  rhythm,  arid 
constancy;  whether  they  are  irregular,  purposive  or  spasmodic ; 
the  muscles  affected  and  the  influence  of  rest,  action,  or 
emotion  upon  them,  and  whether  they  are  under  the  control 
of  the  will. 

3.  The  power  of  the  various  groups  of  muscles.  This 
includes  the  range  as  well  as  the  strength  of  muscular  action. 
The  patient  should  be  asked  to  perform  the  ordinary  move- 
ments of  the  limbs,  trunk,  and  head.  Should  any  weakness  or 
inability  to  perform  them  be  noted,  the  examiner  should  feel 
the  muscles  during  their  attempt  at  movement,  with  a  view  to 
ascertain  whether  they  are  really  contracting.  The  state  of 
the  antagonistic  muscles  should  also  be  noted,  as  in  cases 
of  functional  paralysis  the  contraction  of  the  antagonistic 
muscles  can  be  felt  before  that  of  the  prime  movers. 

In  testing  the  defects  of  the  muscular  power,  it  is  important 
to  remember  that,  in  cerebral  paralysis,  the  loss  of  power 
is  due  to  paralysis  of  voluntary  movement,  while  in  spinal  and 
peripheral  paralysis,  the  loss  of  power  depends  upon  paralysis 
of  individual  muscles  or  groups  of  muscles. 

Thus  in  hemiplegia  (cerebral  paralysis),  the  examination 
consists,  in  the  first  place,  in  asking  the  patient  to  perform  all 
the  voluntary  movements,  commencing  with  the  proximal  and 
proceeding  to  the  distal  joints.  The  trunk  movements  should 
also  be  tested.  The  points  to  be  observed  in  this  examination 
are : — 

(a)  The  ability  or  inability  to  perform  the  movements. 

(b)  The  strength  of  the  movements. 

(c)  The  range  of  movement. 

(d)  The  condition  of  the  joints. 

In  the  peripheral  type  of  paralysis,  the  examination  should 
be  conducted  along  similar  lines : — 

(a)  The  patient's  ability  to  perform  movements. 

(b)  Where  inability  exists,  the  action  of  individual  muscles 
concerned  in  any  movement  should  be  investigated.  This 
includes  not  only  the  prime  movers,  but  the  antagonists  and 
the  synergic  muscles. 

(c)  The  degree  of  paralysis  of  the  individual  muscles  is 
ascertained  by  observing  whether  any  movement  is  produced, 
or  whether  any  contraction  can  be  felt. 

The  strength  of  the  muscular  movements  of  the  hand  may 


38  NERVOUS  DISEASE^ 

be  tested  by  means  of  the  dynamometer,  when  a  comparative 
record  is  required  ;  but  the  most  practical  and  efficacious 
procedure  is  to  make  the  patient  perform  movements  against 
the  resistance  of  the  examiner's  hand,  when  a  good  idea  may 
be  obtained  not  only  of  the  power,  but  also  of  the  effort  made 
by  the  patient  to  carry  out  the  movement.  Associated  move- 
ments should  also  be  observed. 

4.  The  condition  and  size  of  the  muscles.  The  examiner 
will  observe  whether  there  is  increase  or  abnormal  development 
of  any  muscles  ;  whether  muscles  are  wasted  or  diminished  in 
size,  and  whether  such  wasting  is  accompanied  by  flaccidity 
or  spasticity ;  whether  fibrillary  tremor  is  present  or  not  ; 
and  whether  any  muscular  contractures  are  giving  rise  to 
deformities  of  the  limb.  It  ought  to  be  noted  also,  whether 
the  deformity  is  due  to  contracture  of  paralysed  muscles  — 
as  in  old-standing  facial  palsy  ;  to  over-action  of  non- 
paralysed  antagonistic  muscles — as  in  infantile  paralysis  ;  or 
to  the  over-action  of  the  stronger  muscles — as  in  the  flexor 
contracture  of  an  old-standing  hemiplegia.  The  mechanical 
irritability  of  the  muscles,  or  their  contraction  to  direct 
percussion,  should  also  be  noted. 

5.  Muscular  tone.  The  limbs  being  relaxed  should  be 
passively  moved  at  the  several  joints,  and  observations  made 
as  to  whether  the  normal  flexibility  of  the  limbs  is  present. 
In  cases  with  increased  tone  (hypertonia)  some  degree  of 
muscular  resistance  is  felt ;  in  greater  degree,  this  is  known 
as  spasticity,  and  if  severe  it  may  be  so  great  as  not  to  be 
passively  overcome.  In  loss  or  diminution  of  tone  (hypo- 
tonia) the  movements  at  the  joints  are  in  excess  of  normal 
(flaccidity).  In  extreme  degrees  the  limbs  may  be  made  to 
assume  abnormal  positions  as  in  locomotor  ataxia.  As  a 
rule,  hypertonia  is  associated  with  active,  and  hypotonia 
with  absent,  tendon  jerks. 

6.  Co-ordination  of  muscular  action.  This  is  tested  by 
asking  the  patient  to  touch,  for  example,  the  tip  of  the  nose, 
or  to  approximate  the  index  fingers  of  the  two  hands,  to  walk 
along  a  straight  line,  or  to  place  the  heel  of  one  foot  on  the 
knee  of  the  other  leg,  and  so  on.  Finer  movements  may  be 
tested  by  the  act  of  writing.  Tests  should  also  be  applied  with 
the  eyes  closed,  observation  being  made  as  to  whether  this 
induces  or  increases  any  want  of  co-ordination.      Care  should 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        39 

be  taken  to  discriminate  between  the  unsteadiness  caused  by 
tremor  and  muscular  weakness  and  that  due  to  inco-ordination. 

7.  Electrical  reaction.  The  faradic  irritability,  as  being 
the  more  important,  should  first  be  tested.  If  diminished  or 
lost,  the  reaction  to  galvanism  should  be  investigated,  first  as 
regards  the  character  of  the  reaction,  whether  brisk,  feeble,  or 
sluggish;  and  secondly,  as  to  the  polar  reactions. 

(For  a  fuller  account  of  the  electrical  reactions  the  reader 
is  referred  to  p.  50.) 

Examination  op  the  Sensory  System 

The  sensory  system  is  tested  with  a  view  to  the  investi- 
gation of  the  following  points : — 

1.  Whether  sensibility  is  normal,  impaired,  or  lost. 

2.  The  area  or  areas  over  which  impairment  or  loss 

is  detected. 

3.  The  quality  of  the  sensory  impairment  or  loss. 

4.  The  degree  of  the  sensory  impairment. 

5.  The  existence  of  hyperesthesia. 

1.  Is  sensibility  impaired  or  lost  ?  A  routine  examina- 
tion should  be  made  in  every  case,  including  the  following 
tests :  cotton  wool,  pin-prick,  finger  touch,  deep  pressure, 
localisation  and  sense  of  position.  If  any  of  these  sensibilities 
is  defective  or  lost,  a  more  minute  and  detailed  investigation 
should  be  undertaken. 

In  cases  where  the  patient's  mental  condition  precludes  a 
full  examination,  such  tests  ought  to  be  employed  as  will  give 
an  approximate  idea  of  the  state  of  the  sensory  functions. 

2.  The  areas  of  defective  sensibility .  The  examination 
will  show  whether  the  defects  are  universal,  unilateral,  limited 
to  one  extremity,  to  a  segment  or  portion  of  a  limb,  irregular 
or  crossed,  bilateral  without  inclusion  of  the  face,  extending 
up  to  a  definite  segmental  level,  or  bilateral  affecting  the 
extremities  or  segments  of  the  limbs,  or  whether  the  defects 
are  of  root  or  nerve  distribution. 

3.  The  quality  of  the  sensory  loss.  The  recent  work 
of  Head  has  shown  that  the  various  forms  of  sensibility  are 
grouped  differently  in  the  peripheral  nerves  and  spinal  cord 
(p.  14).  In  some  cases  the  lesion  may  be  obviously  spinal 
or  peripheral,  when  the  investigation  of  sensibility  should  at 
once  be  undertaken  along  the  appropriate  lines.     The  only 


40  NERVOUS  DISEASES 

routine  method  of  investigation  is  the  examination  of  every 
form  of  sensibility.  We  will  therefore  describe  separately  the 
method  of  testing  each. 

1.  Cutaneous  sensibility,  including: — 

(a)  Tactile  sensibility; 

(b)  Discrimination  of  the  two  points  of  a  compass  ; 

(c)  Moderate  degrees  of  heat  and  cold ; 

(d)  Cutaneous  pain  ; 

(e)  Extremes  of  heat  and  cold. 

2.  Deep  sensibility,  including  : — 

(a)  Contact  or  pressure ; 

(b)  Painful  pressure  ; 

(c)  Sense  of  position,  passive  and  active  ; 

(d)  Vibration. 

3.  Localisation. 

4.  Stereognosis. 

I.  Cutaneous  sensibility 

Tactile  sensibility.  This  is  tested  b}r  means  of  cotton 
wool  rolled  to  a  fine  point.  The  patient  should  be  shown 
what  the  testing  medium  is,  and  then  told  to  close  his  eyes 
and  indicate  when  and  where  he  is  touched.  Where  the 
epidermis  is  thick,  or  the  sensibility  generally  of  a  low  order, 
a  camel's-hair  brush  may  be  substituted  for  the  cotton  wool. 

Tactile  discrimination.  This  is  tested  by  means  of  the 
blunt  points  of  a  compass  separated  from  each  other  at 
known  intervals.  As  this  form  of  sensibility  varies  widely  in 
different  localities,  it  is  necessary  to  apply  the  test  according 
to  the  normal  standard  of  the  region. 

Care  must  be  taken  to  apply  the  two  points  simultaneously, 
and  a  record  obtained  from  a  given  area  by  touching  the 
patient  a  definite  number  of  times.  Head  has  constructed  a 
graphic  formula  by  using  the  number  of  times  touched  by 
the  double  points  as  the  numerator,  and  by  the  single  points 
as  the  denominator.  The  distance  between  the  compass-points 
must  be  kept  constant  during  each  test. 

It  is  advisable,  where  possible,  to  use  the  corresponding 
skin  area  as  the  normal  standard  or  control. 

Moderate  degrees  of  temperature.  Considerable  varia- 
tions exist  in  different  persons  as  to  what  are  moderate 
shades  of  temperature  ;    but  a  temperature   between  25°  C. 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        4 1 

and  40°  C.  may  be  employed  for  the  purposes  of  this  test. 
Test  tubes  containing  water  of  known  and  constant  tempera- 
tures may  be  employed,  and  the  patient,  with  his  eyes  closed,  is 
asked  to  state  whether  touched  with  the  warm  or  the  cold  tube. 

Cutaneous  pain.  In  testing  this,  care  ought  to  be  taken 
not  to  exercise  pressure.  It  may  be  tested  either  by  gently 
pulling  the  hairs,  or  by  using  the  point  of  a  steel  pin,  which 
should  not  be  too  sharp.  The  patient  is  asked  to  state 
whether  the  pin-point  communicates  a  painful  sensation  or 
not.  He  should  not  be  asked  to  say  whether  the  head  or 
the  point  of  the  pin  is  used,  because,  if  tactile  sensibility  is 
preserved,  it  is  easy  to  discriminate  between  them.  If 
painful  sensibility  is  impaired  or  abolished,  the  pin-point  is 
recognised  merely  as  a  tactile  impression.  Any  delay  in  the 
appreciation  of  pain  should  be  noted. 

Extremes  of  heat  and  cold.  (Heat  above  45°  C.  and  cold 
below  20°  C.)  The  patient  should  be  asked  whether  he  re- 
cognises them,  whether  they  give  rise  to  painful  impressions, 
or  whether  they  are  confused  with  each  other. 

2.  Deep  sensibility 

Contact  or  light  pressure.  For  testing  this  a  stimulus 
sufficient  to  produce  displacement  of  the  skin  is  required — 
such  as  the  application  of  the  finger  to  the  surface  of  the  skin. 

Deep  pain  or  painful  pressure.  Pressure  exerted  with 
sufficient  force  gives  rise  to  a  painful  sensation  in  the  deeper 
tissues — such  as  muscles  or  bones.  This  is  tested  by  pinching 
the  muscles. 

For  the  measurement  of  painful  pressure,  Cattell  has 
devised  an  instrument — the  algometer — which,  by  means  of  a 
spring  and  a  graduated  scale,  records  the  amount  of  pressure 
applied. 

Sense  of  position — passive  and  active.  This  is  a  com- 
plex form  of  sensibility.  In  testing  passive  position,  the  limb 
is  relaxed  and  the  joints  moved  passively,  care  being  taken  that 
in  effecting  these  movements,  only  the  lateral  surfaces  of  the 
limb  are  grasped,  as  this  reduces  to  a  minimum  the  aid  given 
by  cutaneous  impressions.  The  patient,  whose  eyes  are  closed, 
is  then  asked  to  state  the  position  in  which  he  thinks  the 
limb  is  placed  ;  or  he  may  be  asked  to  place  the  corresponding 
limb  in  the  like  position.     In  doubtful  cases  the  patient  may 


42  NERVOUS  DISEASES 

be  asked  to  look  at  the  position  of  the  limb  and  to  state 
whether  the  position  is  what  he  imagined  it  to  be.  The  joint 
sensibility  is  that  concerned  mainly  in  passive  movements  of 
the  limbs. 

In  testing  the  sense  of  movement  (active  position)  the 
patient  is  asked  to  carry  out  some  definite  movement,  such  as 
approximating  the  points  of  the  index  fingers,  or  touching  the 
nose  with  the  finger,  and  the  deviation  or  error  observed.  In 
uncomplicated  cases,  this  is  some  measure  of  the  amount  of 
interference  with  the  sense  of  movement  and  of  the  joint 
sense.  But  as  this  test  calls  into  play  motor  and  cerebellar 
functions,  the  examination  of  the  sense  of  passive  position 
alone  is  to  be  preferred. 

Vibration.  This  is  tested  by  means  of  a  large  tuning-fork 
placed  upon  the  bones  of  the  limbs,  particularly  over  certain 
prominences  — such  as  the  styloid  prominences,  the  malleoli 
or  the  sternum.  The  patient  is  asked  to  state  whether  he 
feels  the  vibration. 

3.  Localisation 

In  testing  localisation  it  is  important  to  ask  the  patient,  if 
possible,  to  indicate  the  spot  touched.  In  certain  cases  it  is 
permissible  to  allow  him  to  look  at  the  limb  before  he  points 
to  the  spot.  The  errors  of  localisation  should  be  noted : 
whether  distal  or  proximal,  pre-  or  post-axial.  Erroneous 
recognition  as  regards  the  limb,  or  side  of  the  body 
(allocheiria)   should  also  be  noted. 

4.  Stereognosis 

This  is  the  ability  to  recognise  without  aid  of  vision  the 
shape,  size,  and  consistence  of  objects  placed  in  the  hands. 
Care  should  be  taken  not  to  diagnose  astereognosis  unless  all 
forms  of  primary  sensibility  are  retained  (p.  251). 

Examination  of  the  Reflexes 

The  reflexes  are  of  three  kinds  :  the  superficial,  obtained 
by  stimulation  of  the  skin ;  the  deep,  obtained  by  striking  the 
tendon  of  a  muscle  under  slight  tension  ;  and  the  organic, 
which  are  concerned  in  swallowing,  micturition,  and  de- 
falcation. 

The   examination  of  the  reflexes  is  of  importance  in  two 


EXAMINATION  OF  THE  NERVOUS  SYSTEM        43 

ways  :  first,  because  their  deviation  from  the  normal  may  be 
direct  evidence  of  organic  disease  of  the  nervous  system  ;  and 
second,  because  such  deviation  may  indicate  the  site  of  the 
lesion. 

In  the  normal  healthy  state,  reflex  action  is  unimpaired. 
Alterations  in  the  reflexes  may  either  be  in  the  direction  of 
increase  or  diminution. 

Superficial  reflexes.  Exaggeration  of  these  reflexes  may 
be  found  in  neurotic  persons,  and  in  conditions  of  hyper- 
esthesia, but  is  not  significant  of  organic  disease.  On  the 
other  hand,  impairment  or  abolition  of  the  reflexes  is  of 
great  importance.     The  superficial  reflexes  are  lost  in  : — 

1.  Local  abdominal  conditions — such  as  a  fat  or  pendulous 
belly. 

2.  Local  inflammatory  conditions  of  the  abdominal 
organs. 

3.  Local  injuries  of  nerves  and  root  lesions,  implying  an 
interference  with  the  reflex  arc. 

4.  Some  hysterical  conditions,  especially  in  association 
with  hysterical  anaesthesia.  In  our  experience  loss  of  the 
superficial  reflexes  is  uncommon  in  functional  conditions,  and 
is  more  characteristic  of  an  organic  lesion  of  the  pyramidal 
tracts. 

5.  Organic  affections  of  the  pyramidal  system. 

It  is  important  to  note  in  all  cases,  whether  the  alteration 
in  these  reflexes  is  uni-  or  bilateral,  and  whether  the  impair- 
ment is  associated  with  changes  in  the  deep  reflexes. 

For  example,  in  organic  hemiplegia,  the  superficial  reflexes 
— especially  the  abdominal  and  epigastric — are  diminished  or 
lost  on  the  hemiplegic  side.  This  is  associated  with  a  definite 
increase  of  the  deep  reflexes  upon  the  same  side,  and  by  an 
alteration  in  the  character  of  the  plantar  reflex,  which  tends 
to  revert  from  the  normal  flexor  to  an  extensor  type. 

In  cases  of  compression  paraplegia,  for  example,  at  the 
level  of  the  ninth  dorsal  segment,  the  superficial  reflexes  will 
be  found  normal  above  that  level,  but  abolished  below. 
This  abolition  will  be  associated  with  an  increase  of  the 
deep  reflexes  and  an  extensor  plantar  response  on  both  sides. 

The  superficial  reflexes  commonly  examined  are  : — 

Corneo- conjunctival:  elicited  by  touching  lightly,  or  blowing 
upon  the  cornea.     The  result  is  closure  of  the  eyelids 


II  NERVOUS   DISEASES 

Pharyngeal',  elicited  by  touching  the  posterior  wall  of  the 
pharynx,  when  a  contraction  of  the  pharyngeal  muscles  results. 

Palatal :  elicited  by  touching  the  soft  palate.  The  result  is 
retraction  of  the  soft  palate  and  drawing  up  of  the  uvula. 

Scapular  :  elicited  by  stroking  the  skin  between  the  shoulder 
blades.     The  result  is  contraction  of  the  scapular  muscles. 

Bulbo-cavernosus :  elicited  by  pinching  the  dorsum  of  the 
penis.  The  result  is  contraction  of  the  bulbous  part  of  the 
urethra. 

Anal  :  pricking  the  skin  over  the  perinseum  evokes 
contraction  of  the  anal  sphincter. 

Epigastric  and  abdominal:  obtained  by  gently  stroking 
the  skin  of  the  abdomen  along  the  costal  margin  and  the 
outer  border  of  the  rectus  abdominalis  muscle. 

Cremasteric :  tested  by  stroking  the  skin  along  the  inner 
side  of  the  thigh.     The  result  is  drawing  up  of  the  testicle. 

Gluteal :  by  stroking  the  skin  along  the  gluteal  fold,  the 
gluteal  muscles  contract. 

The  plantar  reflex.  In  conditions  of  health,  stroking  the 
outer  margin  of  the  sole  of  the  foot,  or  across  the  balls  of 
the  toes,  produces  inversion  of  the  foot,  flexion  of  the  small 
toes,  and  of  the  big  toe  at  the  metatarso-phalangeal  joint, 
and  sometimes  flexion  of  the  thigh  at  the  hip. 

In  testing  this  reflex  it  is  essential  to  have  the  leg  slightly 
everted  at  the  hip  joint,  flexed  at  the  knee,  and  relaxed  at  the 
ankle  joint.  The  sole  of  the  foot  also  should  be  warm  and 
free  from  moisture.  The  important  feature  of  this  reflex  is 
the  movement  of  the  great  toe.  A  voluntary  withdrawal 
or  twitching  of  the  foot,  which  occurs  in  sensitive  persons, 
should  not  be  mistaken  for  a  reflex  action.  The  variations 
from  the  normal  commonly  observed  are  of  two  kinds  :  — 

1.  Abolition  of  all  movement.  This  occurs  in  coma,  in 
certain  Irysterical  conditions  ;  in  lesions  of  the  reflex  arc  of  the 
first  sacral  segment,  with  the  sensory  disturbances  involving 
the  sole  of  the  foot  in  tabes ;  with  paralysis  of  the  muscles 
subserving  the  toes  in  acute  poliomyelitis  ;  in  complete 
transverse  lesions  of  the  cord,  and  when  the  feet  are  cold. 

2.  Extensor  response,  or  extension  of  the  bif/  toe.  The 
substitution  of  an  entirely  different  action,  characterised  by 
eversion  of  the  foot  and  extension  of  the  great  toe  is  direct 
evidence  of  interference  with  the  pyramidal  system.      It  is 


EXAMINATION  OF  THE  NERVOUS  SYSTEM       45 

important  to  realise  that  such  extensor  response  is  not  a  loss 
of  a  superficial  reflex,  but  merely  a  reversion  to  the  normal 
reflex  obtained  in  infants  before  they  have  learned  to  walk, 
and  before  the  pyramidal  fibres  have  received  their  medullary 
sheaths. 

The  paradoxical  plantar  reflex1  is  elicited  by  pressing 
deeply  on  the  calf  muscles,  the  patient  lying  on  his  back  with 
the  muscles  of  the  leg  relaxed.  The  result  is  an  extensor  move- 
ment of  all  the  toes.  This  reflex  is  sometimes  obtained  when 
the  plantar  response  is  flexor,  but  is  not  present  in  health. 

Oppenheim's  reflex  is  elicited  by  pressing  the  thumb  upon 
the  leg  just  behind  the  inner  border  of  the  upper  end  of  the 
tibia  and  firmly  moving  it  towards  the  ankle.  In  normal 
cases  the  foot  is  inverted  and  the  great  toe  flexed ;  but  in 
lesions  of  the  upper  motor  neurone  the  foot  may  be  everted 
and  the  great  toe  extended.  Extension  may  occur  in  cases 
where  the  plantar  response  is  indefinite  or  even  flexor. 

Deep  reflexes.  A  mere  increase  of  the  deep  or  tendon 
reflexes  may,  or  may  not,  be  of  significance.  Exaggeration 
is  significant  of  organic  disease  (1)  if  it  is  present  upon  one 
side,  (2)  if  below  a  definite  level  (the  reflexes  above  remaining 
normal),  (3)  if  associated  with  spasticity,  or  with  changes  in 
the  superficial  and  plantar  reflexes. 

Loss  of  the  deep  reflexes  is  of  great  clinical  importance,  for 
it  is  practically  certain  that  this  phenomenon  is  never  present 
as  a  normal  condition.  Their  abolition  therefore  indicates 
organic  affection  of  some  part  of  the  reflex  arc — either  the 
afferent  limb,  the  motor  cells  of  the  anterior  horns,  the  efferent 
limb,  or  the  muscles.  They  are  also  abolished  temporarily  in 
acute  lesions  of  the  cerebellum,  and  in  complete  transverse 
lesions  of  the  spinal  cord  above  the  lumbar  enlargement. 

The  deep  reflexes  are  tested  by  placing  the  limb  in  such  a 
position  as  to  relax  the  muscle  whose  tendon  jerk  is  to  be 
elicited.  The  muscle  is  then  put  under  slight  tension,  and  by 
tapping  the  tendon  sharply  with  the  finger  or  a  small  hammer, 
a  muscular  contraction  of  greater  or  less  degree  results.  This 
test  really  indicates  the  state  of  the  muscular  tone,  which 
depends  in  large  part  upon  the  integrity  of  the  reflex  arc. 

The  deep  reflexes  commonly  examined  and  the  methods  of 
testing  are  as  follows  :  — 

1  Gordon,  American  Medicine,  1904. 


46  NERVOUS  DISEASES 

The  jaw  jerk.  The  patient  opens  the  mouth,  and  the 
examiner  slightly  depressing  the  chin  with  his  finger,  so  as  to 
stretch  the  masseter  muscles ;  taps  the  jaw,  thereby  inducing 
a  contraction  of  the  masseter  muscles. 

Scapular.  Tapping  the  vertebral  border  of  the  scapula 
causes  a  retraction  of  the  shoulder. 

Triceps.  The  arm  is  semi-flexed  at  the  elbow  joint,  and 
the  tendon  is  struck  just  above  its  insertion  into  the 
olecranon  process. 

Biceps.  This  is  obtained  by  flexing  the  elbow  joint,  placing 
the  thumb  upon  the  tendon  of  the  biceps  and  slightly  stretch- 
ing the  muscle.  The  thumb  is  then  struck  and  the  biceps 
contracts  sharply. 

Supinator  and  ivrist  jerks.  The  arm  is  supported  with 
the  elbow  slightly  flexed  and  the  radius  struck  just  above 
the  styloid  process. 

Knee  jerk.  The  patient  is  seated  on  a  chair  with  his  feet 
upon  the  floor.  The  knee  being  slightly  flexed  so  as  to  relax 
the  rectus  femoris  muscle,  a  contraction  of  the  quadriceps  is 
obtained  on  striking  the  patellar  tendon.  The  simplest  and 
most  satisfactory  method  of  reinforcing  the  knee  jerk  is  to 
make  the  patient,  when  seated  on  a  chair,  extend  the  leg 
with  the  heel  and  toe  remaining  upon  the  floor.  When  the 
maximum  extension  in  this  position  has  been  reached,  he  is 
asked  to  press  the  toes  firmly  on  to  the  floor  without  raising 
his  heel,  and  the  tendon  is  sharply  struck. 

The  knee  clonus  is  obtained  best  by  extending  the  leg  with 
the  patella  freely  movable.  The  tendon  is  then  put  upon  the 
stretch  by  suddenly  depressing  the  patella. 

Ankle  jerk.  The  examiner  grasps  the  foot,  the  ankle  joint 
being  relaxed,  and  the  leg  everted  at  the  hip  and  flexed  at  the 
knee.  The  tendo  Achilles  is  stretched  by  slightly  dorsiflexing 
the  foot.     The  tendon  is  then  sharply  struck. 

Should  this  method  fail,  the  patient  is  made  to  kneel  upon 
a  chair  and  the  tendon  struck.  Reinforcement  is  obtained 
by  asking  the  patient  to  let  his  body  sink  down  so  that  the 
buttocks  approximate  to  the  heels.  By  this  method  the 
hamstrings  and  calf  muscles  are  relaxed. 

Ankle  clonus.  This  is  obtained  with  the  leg  in  the  position 
for  the  Achilles  jerk,  the  foot  is  grasped  by  the  examiner's 
hand  and  suddenly  dorsiflexed. 


EXAMINATION  OF  THE  NERVOUS  SYSTEM       47 

Organic  reflexes.     Those  usually  examined  are  :  — 

1.  Vesical.  The  patients  should  be  asked  whether  they 
have  any  difficulty  in  passing  or  in  holding  their  water ; 
whether  they  have  any  delay  or  hesitancy  in  the  act  of 
micturition.  If  there  is  any  difficulty  in  passing  water,  local 
conditions  (urethral,  vesical  and  urinary)  should  be  excluded. 

If  the  patients  complain  of  frequency  of  micturition  or 
inability  to  hold  their  water,  the  examination  should  be  made 
to  determine  (1)  whether  there  is  distension  of  the  bladder,  or 
(2)  whether  there  is  loss  of  sphincter  tone,  or  (3)  whether 
there  is  loss  of  control  over  the  sphincter. 

In  women  incontinence  of  urine,  especially  on  sudden  effort, 
may  be  due  to  local  causes. 

In  all  cases  of  nervous  disease,  where  there  is  any  possibility 
of  interference  with  the  sphincter  control,  the  physician  should 
not  be  satisfied  with  the  patient's  statements,  but  should 
himself  determine— by  percussion,  or  if  necessary  by  passing 
a  catheter — whether  the  bladder  is  distended. 

2.  Rectal.  Inquiry  should  be  made  as  to  the  existence  of 
constipation,  or  loss  of  control  over  the  action  of  the  bowels. 
In  the  latter  case,  the  state  of  the  anal  sphincter  should  be 
digitally  examined,  so  as  to  ascertain  whether  it  contracts. 
If  the  anus  is  patulous,  there  is  interference  with  the  reflex 
arc  through  the  fourth  and  fifth  sacral  segments.  If  loss  of 
control  only  occurs  in  the  administration  of  purgatives,  an 
impairment  of  cerebral  control  over  the  spinal  reflex  is 
indicated. 

3.  Swallowing.  This  is  examined  by  testing  the  patient's 
ability  to  swallow  fluids,  semi-solids  and  solids. 

4.  Sexual.  In  certain  cases  it  may  be  necessary  to  make 
inquiry  as  to  the  functions  of  the  sexual  organs ;  whether 
erection  is  possible  or  not,  and  ejaculation  premature,  delayed, 
or  absent. 

The  reflexes  affected  in  association  with  the  sympathetic 
nervous  system  are  : — 

Cilio-spinal.  Pinching  the  skin  of  the  neck  provokes  dilata- 
tion of  the  pupil. 

Scrotal.  Cold  applied  to  the  scrotum  induces  contraction 
of  the  dartos  muscle. 

Vesical,  rectal,  uterine.  Stimulation  of  the  bladder,  rectum 
and  uterus  result  in  contraction  of  the  bladder,  rectum  and 


48  NERVOUS  DISEASES 

uterus.  These  reflexes  may  take  place  independently  of  the 
central  nervous  system.  They  may  be  initiated  by  voluntary 
effort. 

Internal  anal.  A  finger  passed  into  the  rectum  is  gripped 
by  the  contraction  of  the  internal  sphincter  muscle. 

Having  completed  the  examination  of  the  nervous  system, 
the  other  systems  should  be  examined  seriatim.  The  absence 
of  signs  of  organic  disease  of  the  nervous  system  makes  this 
the  more  necessary,  as  nervous  symptoms  may  be  the  earliest 
expression  of  derangement  or  disease  of  some  other  part  of 
the  body.  Disease  of  the  nervous  system  may  be  directly 
dependent  upon  disease  of  other  systems,  especially  the  cardio- 
vascular and  urinary.  Disease  of  the  nervous  system  may 
also  exist  synchronously  with,  but  independent  of  disease  of 
other  systems. 

LuMBAE    PUNCTUEB  :    THE    CeEEBEO-SPINAL    FLUID 

The  examination  of  the  cerebro-spinal  fluid  is  of  consider- 
able value  in  diagnosis.  The  fluid  is  obtained  by  a  lumbar 
puncture.  For  this  purpose  the  patient  is  placed  upon  his 
left  side,  or  sits  in  a  chair,  with  his  back  exposed  to  the 
operator ;  in  either  case  the  head  and  shoulders  are  bent  well 
forwards.  The  space  between  the  larninae  of  the  fourth  and 
fifth  lumbar  vertebrae  is  preferably  chosen  for  the  insertion 
of  the  needle,  on  account  of  the  absence  of  the  spinal  cord  in 
this  situation.  The  needle  is  passed  in  a  slightly  upward  and 
inward  direction  about  half  an  inch  to  one  side  of  the  mesial 
line,  and  pushed  on  until  the  point  is  felt  to  be  free.  The 
fluid  is  allowed  to  escape  drop  by  drop  to  the  extent  of 
3  to  5  c.cm.  The  needle  is  then  withdrawn  and  the  wound 
covered  with  a  collodion  dressing. 

If  the  puncture  is  effected  under  strict  antiseptic  precau- 
tions and  the  patient  kept  in  bed  for  twenty-four  hours, 
no  risk  attends  this  operation.  In  many  cases,  however, 
even  when  precautions  are  taken,  the  patient  may  experience 
headache  of  a  peculiarly  disagreeable  type  when  he  sits 
up.  This  may  depend  upon  the  quantity  taken  away,  which 
should  not  exceed  5  c.cm.  In  cases  of  intracranial  tumour 
with  great  increase  of  intracranial  pressure  there  is  a  risk 
of  sudden  collapse  and  death. 


EXAMINATION  OF  THE  NERVOUS  SYSTEM       49 

Two  methods  are  used  for  the  cytological  examination  of 
the  fluid.  A  known  quantity  is  centrifugalised  for  a 
definite  time,  some  of  the  deposit  transferred  to  a  slide, 
fixed  and  stained,  and  the  number  of  cells  counted  under 
the  field  of  the  objective.  In  the  other  method  the 
fluid  is  handled  as  in  the  counting  of  blood,  a  few 
drops  being  placed  in  the  cell  of  a  Thomas- Zeiss  slide, 
and  the  number  of  cells  counted  and  estimated  per  cubic 
millimetre. 

The  normal  fluid  is  a  clear  watery  liquid  of  low  specific 
gravity  and  alkaline  reaction,  and  contains  a  small  per- 
centage of  chlorides,  some  serum  globulin,  a  trace  of  cholin, 
and  a  substance  which  reduces  Fehling's  solution. 

Albumen  is  not  normally  present,  but  may  be  found  in 
considerable  quantity  in  cases  of  meningitis. 

The  quantity  of  cholin  normally  present  may  be  increased 
in  organic  nervous  disorders — a  fact  which  has  been  used 
in  the  differential  diagnosis  of  otherwise  doubtful  cases  of 
organic  and  functional  disease. 

The  cytology  of  the  cerebrospinal  fluid.      Only  two  or 
three  lymphocytes  are  found  in  the  centrifuged  deposit  of 
normal   fluid.      In   many   pathological   conditions,  the  cells 
are  materially  increased.     They  are  of  two  kinds  : — 
(a)  Lymphocytes  or  mono-nucleated  cells. 
•   (b)  Polymorpho-nuclear  cells. 

The  lymphocytes  are  about  the  size  of  a  red  blood 
corpuscle.  They  contain  one  large  deeply  staining  nucleus, 
surrounded  by  a  rim  of  protoplasm. 

The  polymorpho-nuclear  cells  are  larger,  with  a  lobulated 
or  horse-shoe  shaped  nucleus,  and  a  large  amount  of 
protoplasm  containing  a  fine  granular  material. 

In  addition,  large  endothelial  cells  may  be  seen,  containing 
an  irregularly  shaped  nucleus  and  much  protoplasm. 

The  factors  which  determine  a  mono-  or  polymorpho- 
nuclear exudation  are  not  sufficiently  known,  but  it  would 
seem  as  if  the  former  is  the  effect  of  a  toxic  condition,  while 
the  latter  is  attributable  to  an  acute  inflammatory  process 
associated  with  the  presence  of  micro-organisms. 

An  increase  of  the  lymphocytes  is  found  in  cerebro- 
spinal syphilis,  in  the  parasyphilitic  diseases — tabes  dorsalis 
and   general   paralysis — in  the  early   stages    of  tuberculous 

4 


50  NERVOUS  DISEASES 

meningitis,  in  post-basic  meningitis,  and  in  the  convalescent 
or  chronic  stage  of  cerebro-spinal  fever. 

The  polymorpho-nuclear  cells  are  found  in  the  acute  forms 
of  meningitis,  including  the  rapid  cases  of  tuberculous 
meningitis,  and  in  cerebro-spinal  fever  during  the  acute 
period  of  the  symptoms. 

The  bacteriology  of  the  cerebro-spinal  fluid.  A  bacterio- 
logical examination  should  be  carried  out  in  conjunction  with 
the  cytological.  The  presence  of  a  specific  micro-organism 
confirms  a  diagnosis  which  may  have  been  still  doubtful  from 
the  cell  examination  alone. 

Electrical  Examination 

Normally  a  contraction  is  obtained  when  an  electrode, 
attached  to  either  a  faradic  or  a  galvanic  battery,  is  applied 
to  a  muscle  and  the  circuit  closed.  As  this  reaction  may  be 
modified  or  lost  in  certain  diseases  of  the  nervous  system,  the 
electrical  reactions  of  the  muscles  are  of  some  importance  in 
the  clinical  examination  of  a  case  in  which  muscular  weakness 
or  atrophy  is  present. 

Reactions  in  health 

1.  Faradic  contractility.  The  faradic  contractility  of  a 
muscle  is  obtained  by  applying  the  electrode  over  the  muscle 
to  be  tested,  more  especially  over  the  motor  point  or  that 
portion  of  the  muscle  into  which  the  motor  nerve  passes. 
The  normal  reaction  is  a  brisk,  well-sustained  muscular 
contraction. 

2.  Galvanic  contractility.  Muscular  contraction  is  only 
obtained  when  the  circuit  is  closed  and  opened  ;  none  is 
observed  during  the  continued  flow  of  the  current.  As  there 
are  two  poles,  four  series  of  contractions  may  be  observed  : 
two  at  closing  of  the  circuit  (positive  and  negative  poles)  and 
two  at  opening  of  the  circuit  (positive  and  negative  poles). 
These  are  represented  usually  by  letters,  thus  : — ■ 

K.C.C.  =  kathodal  closing  contraction. 
A.C.C.  =  anodal  closing  contraction. 
A.O.C.  =  anodal  opening  contraction. 
K.O.C.  =  kathodal  opening  contraction. 
The  above  order  is  that  found  in  health,  but  owing  to  the 


EXAMINATION  OF  THE  NERVOUS  SYSTEM       51 

very  strong  current  required  to  give  the  opening  contractions 
their  examination  is  usually  dispensed  with. 

The  reaction  in  health  may  therefore  be  stated  as  follows  : — 
Nerve.     Eeaction  to  faradism  and  to  galvanism. 
Muscle.  Eeaction  to  faradism,  brisk  and  well  sustained. 
Reaction  to  galvanism,  short  and  sharp. 

K.C.C.  >  A.C.C.  :  A.O.C.  >  K.O.C. 

Reactions  in  disease  of  the  lower  motor  neurone 

In  certain  states  of  muscular  wasting — lesions  of  the 
lower  motor  neurone — an  important  alteration  in  the  electrical 
irritability  of  nerve  and  muscle  is  obtained,  known  as  the 
reaction  of  degeneration.  This  consists  of  a  loss  or  impair- 
ment of  the  faradic  irritability,  in  association  with  a  modi- 
fication of  the  contraction  to  galvanism,  both  quantitatively 
and  qualitatively.  The  quantitative  change  is  found  in  the 
replacement  of  the  short,  sharp  reaction  of  health  by  a  slow 
and  sluggish  muscular  response.  The  qualitative  change  is 
seen  in  the  equality  of  the  K.C.C.  and  A.C.C,  or  the  more 
ready  response  of  the  A.C.C.  If  the  strong  current  can 
be  borne,  the  K.O.C.  will  be  obtained  more  easily  than  the 
A.O.C. 

The  reaction  of  degeneration  (R.D.)  is  therefore  as 
follows  :  — 

Nerve.     Reaction  to  both  faradism  and  galvanism  lost. 
Muscle.  Reaction  to  faradism  impaired  or  lost. 

Reaction  to  galvanism,  slow  and  sluggish. 

A.C.C.  >  K.C.C.  ;  or  K.C.C.  =  A.C.C. ;  K.O.C.  >  A.O.C. 

All  degrees  of  incompleteness  are  seen  from  mere  diminu- 
tion of  faradic  excitability  to  the  fully  developed  reaction  of 
degeneration. 

The  state  of  the  electrical  reactions  is  a  guide  to  prognosis. 

Reactions  in  other  diseases 

In  certain  diseases — the  myopathies,  myasthenia  gravis, 
tetany,  and  myotonia  congenita  (Thomsen's  disease),  the 
electrical  reactions  show  distinctive  and  characteristic  changes, 
which  are  detailed  later  in  the  descriptions  of  these  disorders. 


L\\HT    III 


THE    SPECIAL   SENSES 

SMELL 

The  olfactory  system  consists  of:  (a)  the  olfactory  bulb, 
into  which  the  olfactory  nerves  from  the  Schneiderian  mucous 
membrane  enter  through  the  cribriform  plate  of  the  ethmoid 
bone,  (b)  The  olfactory  tract,  which  divides  into  two  roots — an 
external  passing  towards  the  apex  of  the  temporal  lobe  and  the 
hippocampal  lobule,  and  a  mesial  which  crosses  to  the  opposite 
side  by  the  anterior  commissure,  (c)  A  cortical  centre,  which 
appears  to  be  located  in  the  lobus  pyriformis  or  uncinate 
gyrus,  and  to  some  extent  in  the  anterior  part  of  the  gyrus 
fornicatus. 

Anosmia,  or  loss  of  the  sense  of  smell,  is  a  rare  symptom 
of  nervous  disease.  It  is  due  more  commonly  to  disease 
within  the  nose. 

It  may  arise  from  the  following  causes  : — 

(a)  Local  conditions  within  the  nasal  cavities — such  as 
enlargement  of  the  turbinated  bones,  polypi,  and  foetid 
atrophic  rhinitis. 

(b)  Arrest  of  secretion  and  '  trophic  '  changes  in  cases  of 
paralysis  of  the  trigeminal  nerve. 

(c)  Fracture  of  the  base  of  the  skull  involving  the  olfactory 
bulb  or  tract  in  the  anterior  fossa. 

(d)  Local  intracranial  disease — such  as  meningitis,  tumour 
of  the  frontal  lobe  pressing  upon  the  olfactory  tract,  and 
tumours  of  the  pituitary  region. 

(e)  Hysteria,  in  association  with  other  hysterical  stigmata, 
such  as  hemi-ansesthesia  (p.  527). 

Parosmia,  or  perverted  sense  of  smell,  may  be  found 
in  association  with  local  nasal  disease.      We  have  seen  it  as 

52 


THE  SPECIAL  SENSES  53 

an  isolated  and  troublesome  after-effect  of  influenza.  Sub- 
jective sensations  of  smell  of  central  origin  occur  in  cases  of 
tumour  involving  the  cortical  centre,  situated  in  the  uncinate 
region  of  the  temporo-sphenoidal  lobe.  They  are  also  found 
as  the  aura  in  some  cases  of  idiopathic  epilepsy.  Hallucina- 
tions of  smell  are  occasionally  met  with  in  the  insane. 

Subjective  sensations  of  smell  are  generally  of  an  unpleasant 
character. 

TASTE 

Nerve  fibres  subserving  the .  sense  of  taste  are  distributed 
over  the  tongue,  soft  palate,  and  palatine  arches. 

The  course  of  the  taste-fibres  between  the  periphery  and 
the  brain  is  complicated,  and  is  still  the  subject  of  discussion. 
The  following  facts  appear  to  be  proven  :  (a)  The  taste-fibres 
for  the  anterior  two-thirds  of  the  tongue  are  distributed  in 
the  lingual  branch  of  the  fifth  nerve.  From  this  nerve  the 
major  portion  pass  into  the  chorda  tympani  and  run  along 
with  the  facial  nerve  in  the  aqueductus  Fallopii,  as  far  as 
the  geniculate  ganglion.  From  this  they  pass  directly 
through  the  sensory  root  of  the  seventh  to  the  pons  Varolii. 

Entering  the  medulla  oblongata  by  the  sensory  root  of 
the  facial  nerve,  and  probably  also  by  the  roots  of  the  glosso- 
pharyngeal, the  fibres  of  taste  pass  upwards,  but  do  not 
proceed  along  with  the  other  sensory  fibres  to  the  internal 
capsule.  They  find  their  way  by  an  as  yet  unknown  route  to 
the  anterior  end  of  the  temporo-sphenoidal  lobe,  where  the 
cortical  centre  for  taste  has  been  located  in  close  relation  to 
the  olfactory  centre.  The  histological  researches  of  Campbell 
favour  the  anterior  part  of  the  island  of  Eeil  as  a  probable 
gustatory  cortical  centre. 

(b)  The  taste-fibres,  from  the  posterior  third  of  the  tongue, 
are  distributed  in  the  glosso-pharyngeal  nerve.  Some  fibres 
may  be  transmitted  to  the  brain  by  the  roots  of  this  nerve, 
but  others  course  through  the  nerve  of  Jacobson  and  the 
small  superficial  petrosal  nerve  to  the  otic  ganglion  on  the 
third  division  of  the  fifth  nerve. 

(c)  The  fibres  from  the  soft  palate  and  palatine  arches  pass 
through  the  Vidian  nerve  and  great  superficial  petrosal  to 
the  geniculate  ganglion.     Excision  of  the  Gasserian  ganglion 


54  NERVOUS  DISEASES 

is  only  rarely  followed  by  loss  of  taste  over  the  half  of  the 
tongue,  and  this  is  not  a  permanent  defect. 

The  statistical  observations  of  dishing1  and  Davies,2  based 
upon  the  studies  of  thirty-nine  cases  of  excision  of  the 
Gasserian  ganglion  for  trigeminal  neuralgia,  showed  that  only 
two  of  these  had  loss  of  taste,  four  impaired  sense  of  taste,  and 
thirty-three  no  impairment  of  taste  after  this  operation.  It 
would  therefore  appear  as  if  the  glosso-pharyngeal  roots  and 
the  sensory  root  of  the  facial  were  the  only  transmitters  of 
taste  to  the  brain. 

Ageusia,  or  loss  of  taste,  occurs  in  peripheral  facial 
paralysis,  and  in  hysteria  along  with  herni-anaesthesia  and 
interference  with  the  other  special  senses.  It  may  also  be 
present  in  an  incomplete  form  in  catarrhal  conditions  of  the 
mouth  and  tongue,  and  sometimes  after  influenza.  When 
unilateral  the  patient  may  be  unaware  of  its  existence. 

Parageusia,  or  perverted  taste,  is  present  in  local  disorders 
of  the  mouth,  and  subjective  sensations  of  taste  may  arise 
from  tumours  of  the  uncinate  region. 


VISION 

Anatomy.  The  visual  system  may  be  divided  into  two 
segments  :  a  peripheral,  comprising  the  retina,  optic  nerves, 
chiasma,  optic  tracts  and  their  terminations  in  the  basal 
ganglia — anterior  quadrigeminal  body,  pulvinar  thalami  and 
external  geniculate  body  ;  and  a  central,  consisting  of  the 
nuclei  in  the  pulvinar,  and  external  geniculate  body,  the  optic 
radiations  and  the  cortical  centres  for  sight. 

The  optic  nerves  are  composed  of  three  bundles  of  fibres : 
(a)  a  direct,  or  uncrossed,  bundle  lying  externally,  (b)  a  crossed 
bundle  lying  internally,  and  (c)  a  central,  foveal  or  papillo- 
macular  bundle.  At  the  chiasma  the  crossed  bundles,  which 
come  from  the  nasal  halves  of  the  retina  decussate  ;  but  the 
direct  bundles,  coming  from  the  temporal  halves,  pass  back- 
wards on  the  same  side.  In  consequence  of  this  arrangement 
the  fibres  from  the  homonymous  halves  of  both  retina?  run 
together  in  the  optic  tract.     (Fig.  8.) 

Some  optic  tract  fibres  may  be  traced  into  the  superficial 

1  Cushing,  Bull.  Johns  Hopkins  Hosp.,  1903. 

2  Morriston  Davies.  Brain,  1907. 


THE  SPECIAL  SENSES 


55 


layers  of  the  anterior  quadrigeminal  bodies.  These  probably 
subserve  the  reflex  movements  of  the  pupil  (the  optico-pupil- 
lary  fibres).  The  majority  of  the  optic  tract  fibres  enter  the 
external  geniculate  body  and  the  pulvinar  thalami  optici. 

From   these   ganglia    a   large  mass  of  fibres  is  projected 
towards  the  cortex  of  the  occipital  lobe,  as  the  optic  radiations 


CORP.GEN.INT. 


Fig.  8  (from  Cunningham's  'Anatomy'). — Showing  the  composition 
and  connexions  of  the  fibres  of  the  optic  nerves  and  tracts. 

of  Gratiolet.  In  their  course  towards  the  cortex  these  fibres 
pass  through  the  retrolenticular  portion  of  the  internal  capsule, 
in  juxtaposition  to,  but  behind,  the  sensory  thalamo-  cortical 
fibres  ;  and  farther  on  they  lie  subjacent  to  the  angular  gyrus 
and  in  close  relation  to  the  superior  temporal  convolution, 
mixing  with  the  fibres  of  the  inferior  longitudinal  fasciculus. 


56  NERVOUS  DISEASES 

The  optic  radiations  are  made  up  of  three  distinct  sets  of 
fibres:  (a)  a  bundle  already  medullated  at  birth,  passing  from 
the  external  geniculate  body  to  the  lips  of  the  calcarine  fissure. 
This  forms  the  true  radiation  of  the  visual  fibres  according  to 
Flechsig  and  Henschen. 

(b)  A  system  passing  to  the  adjacent  angular,  cuneal, 
lingual,  and  fusiform  gyri. 

(c)  An  efferent  or  corticifugal  tract,  passing  from  the 
occipital  lobe  to  the  middle  layers  of  the  anterior  quadri- 
geminal  body. 

The  cortical  visual  centres.  Henschen '  has  located  the 
cortical  half-vision,  or  visuo-sensory  centre  on  the  mesial 
aspect  of  the  occipital  lobe  in  the  lips  of  the  calcarine  fissure. 
According  to  von  Monakow  and  others,  this  centre  occupies 
the  lingual  lobe,  the  cuneus,  and  a  portion  of  the  external 
aspect  at  the  occipital  pole.  In  other  words,  the  half- vision 
centre  occupies  that  portion  of  the  lobe  corresponding  to  the 
distribution  of  the  '  line  of  Gennari,'  which  is  in  part  formed 
by  the  fibres  of  the  optic  radiation  and  partly  by  fibres  from 
the  opposite  occipital  lobe  (Mott). 

In  association  with  the  primary  or  half-vision  centre, 
Campbell  and  others  have  described  a  higher,  or  '  psycho- 
visual  '  centre,  occupying  the  external  surface  of  the  occipital 
lobe  and  the  remainder  of  the  cuneus,  and  bounded  in  front 
approximately  by  the  parietooccipital  fissure.  (Figs.  9 
and  11.) 

The  occipital  lobe  has  extensive  connexions  with  other 
portions  of  the  brain,  such  as  the  opposite  occipito-angular 
region  by  way  of  the  splenium  corporis  callosi,  with  the 
frontal  lobe  by  the  superior  longitudinal  fasciculus  and  the 
tapetum,  and  with  the  temporo-angular  region  by  the  inferior 
longitudinal  fasciculus. 

The  last  connexion  is  an  especially  important  one,  as  the 
angular  gyrus,  with  its  subcortical  longitudinal  fasciculus  and 
the  second  occipital  gyrus,  subserve  the  recognition  of  written 
or  printed  language.  This  region  corresponds  to  '  Wernicke's 
area.' 

The  frequent  coexistence  of  homonymous  hemi-anopsia  with 
'  sub-cortical  alexia  '  is  due  to  the  close  relation  which  the 
longitudinal   fasciculus   has   with   the   optic    radiations,    an 

1  Henschen,  Patholog.  des  Geliims,  1892. 


THE  SPECIAL  SENSES  57 

intermingling  of  the  fibres  of  both  structures  taking  place  in 
the  region  of  the  angular  gyrus. 

Physiology.  Owing  to  the  decussation  of  the  fibres  from  the 
corresponding  nasal  halves  of  the  retina  (temporal  fields)  and 
the  uncrossed  position  of  those  from  the  temporal  halves 
(nasal  fields),  each  optic  tract  contains  fibres  from  homo- 
nymous retinal  segments.  The  right  optic  tract,  for  example, 
transmits  the  visual  fibres  from  the  right  halves  of  both 
retinse,  corresponding  to  the  left  homonymous  visual  fields. 
In  this  manner  each  half  of  the  retina  is  represented  in  the 
corresponding  basal  ganglia,  optic  radiation,  and  half-vision 
cortical  centre.     Hence  a  destructive  lesion  of  any  of  these 


Pig.  9  (after  Campbell). — Showing  the  distribution  of  the  cortical  visual 
centre  upon  the  mesial  aspect  of  the  hemisphere.  A,  primary  visual 
centre.     B,  visuo-psychical  centre. 

structures  on  the  right  side  will  occasion,  not  blindness  of  the 
opposite  (left)  eye,  but  blindness  of  the  homonymous  right 
halves  of  both  eyes,  with  loss  of  the  visual  fields  on  the  left 
side.     (Fig.  8.) 

In  homonymous  hemi-anopsia  from  blindness  of  the  corre- 
sponding halves  of  both  retinse  due  to  cerebral  disease,  the 
ine  of  demarcation  rarely  passes  through  the  fixation  point. 
This  point  corresponds  to  the  distribution  of  the  fibres  of  the 
papillo-macular  bundle,  which  we  have  seen  passes  into  both 
optic  tracts. 

The  macular  region  therefore  appears  to  have  a  bilateral 
cortical  representation ;  but  it  is  uncertain  whether  this  lies  in 
the  calcarine  area,  as  believed  by  Henschen,  or  in  a  higher 


58  NERVOUS  DISEASES 

visual  centre  in  the  angular  gyrus,  as  stated  by  Ferrier, 
or  generally  over  the  occipito-angular  cortex,  according  to 
Mxmakow. 

Sector  or  quadrantic  defects  of  the  visual  fields — where 
corresponding  retinal  quadrants  are  rendered  blind,  with  loss  of 
vision  in  the  corresponding  portions  of  the  visual  fields — are 
sometimes  observed.  Beevor  and  Collier  ]  state  that  the  line 
of  separation  between  the  visual  centres  (corresponding  to  the 
upper  and  lower  quadrants  of  the  visual  field)  is  probably  the 
calcarine  fissure ;  the  lower  visual  field  being  represented  in 
the  upper  or  supra-calcarine  area,  and  vice  versa. 

Diseases  of  the  Peripheral  Visual  System 

The  reader  is  referred  to  works  on  ophthalmology  for  an 
account  of  diseases  of  the  retina  and  local  orbital  conditions. 

The  optic  nerve 

A  lesion  of  the  optic  nerve  results  in  impairment  or  loss  of 
vision  upon  the  affected  side. 

The  chief  conditions  affecting  the  optic  nerve  are  optic 
neuritis  and  optic  atrophy. 

Optic  neuritis.  The  common  cause  of  optic  neuritis  is 
intra-cranial  tumour  or  abscess,  especially  when  associated 
with  marked  increase  of  intracranial  pressure.  It  may  also 
be  found  in  renal  disease  (acute  or  chronic),  in  profound 
anaemia,  certain  toxic  states,  lead  encephalopathy,  cerebral 
syphilis,  and  acute  infective  disorders.  It  is  occasionally 
found  in  association  with  acute  myelitis  in  the  cervical  region. 

In  the  early  stages,  blurring  of  the  edges  of  the  disc  and 
hyperemia  of  the  optic  papilla  are  seen.  It  is  important  to 
differentiate  the  early  changes  from  somewhat  similar  appear- 
ances observed  in  some  cases  of  hypermetropia  ;  and  it  should 
be  borne  in  mind  that  a  slight  blurring  of  the  inner  margin  of 
the  disc  is  a  physiological  condition  in  many  eyes.  As  the 
neuritis  increases,  the  hyperemia  and  congestion  become 
more  intense,  the  disc  is  swollen  and  of  a  reddish  colour,  the 
central  cup  is  filled  in,  the  veins  distended  and  the  arteries 
smaller  than  normal.     In  severe  cases  the  swelling  may  be  as 

1  Beevor  and  Collier,  Brain,  1904. 


THE  SPECIAL  SENSES  59 

much  as  7  diopters,  and  the  surrounding  fundus  edematous  ; 
the  vessels  present  the  same  features  as  in  the  earlier  stages, 
but  extensive  exudations  may  be  observed  either  in  patches 
or  extending  along  the  vessels,  and  occasionally  obliterating 
portions  of  them.  Hemorrhages  may  be  seen  scattered  about 
on  the  disc  and  in  its  vicinity,  and  the  general  appearance 
may  resemble  that  of  albuminuric  retinitis.  As  the  swelling 
subsides  the  congestion  lessens,  the  disc  becomes  pale,  but 
the  central  cup  remains  filled  in,  and  newly  formed  connective 
tissue  may  be  seen  surrounding  the  blood-vessels.  The  edges 
of  the  disc  are  blurred  in  places,  and  the  arteries  are  small 
and  tortuous. 

Symptoms.  Vision  may  or  may  not  be  impaired — normal 
vision  being  compatible  with  a  considerable  degree  of  swelling. 
The  earliest  symptoms  are  mistiness  and  blurring  of  sight, 
which  may  at  first  be  intermittent  but  later  become  constant, 
and  associated  with  definite  impairment  of  vision.  In  the 
later  stages,  when  optic  atrophy  has  supervened,  defective 
visual  acuteness  is  present,  and  contraction  of  the  visual  field 
may  be  detected.  Blindness  is  rare  during  the  acute  stage, 
but  supervenes  with  the  advent  of  complete  optic  nerve 
atrophy.  The  disappearance  of  the  pupillary  light  reflex  is 
coincidental  with  the  loss  of  light  perception. 

The  only  treatment  of  any  service  is  the  operation  of 
decompression,  especially  when  optic  neuritis  is  due  to 
intracranial  causes  (p.  277). 

Optic  atrophy  may  arise  as  a  primary  condition  or  follow 
upon  optic  neuritis  or  local  injury  to  the  nerve.  Several 
varieties  of  primary  atrophy  are  described  :  (a)  degenerative 
atrophy — as  in  the  hereditary  type  of  Leber  and  amaurotic 
family  idiocy  ;  (b)  as  a  local  phenomenon  in  general  diseases 
— such  as  tabes  dorsalis,  general  paralysis,  and  disseminated 
sclerosis  ;  (c)  from  local  lesions  of  the  optic  nerve — such  as 
fracture  of  the  base  of  the  skull,  tumour  pressing  directly  on 
the  nerve,  and  retrobulbar  neuritis. 

In  the  primary  optic  atrophy,  which  occurs  in  tabes 
dorsalis,  the  disc  is  grey-white  in  colour  with  clean-cut  edges. 
The  cup  is  not  filled  in,  the  lamina  cribrosa  is  well  seen,  and  there 
is  little  alteration  in  the  calibre  of  the  retinal  blood-vessels.  It 
is  a  bilateral  condition,  progressive  in  its  course,  and  leading 
eventually  to  complete  blindness.     In  some  cases,  however,  a 


60  NERVOUS  DISEASES 

small  central  zone  of  relatively  good  vision  may  persist  for 
a  long  time,  which  would  seem  to  be  due  to  preservation  of  the 
central  or  papillo-macular  bundle  of  optic  nerve  fibres. 

A  form  of  optic  atrophy,  probably  arising  from  the  presence 
of  a  local  sclerotic  patch  in  the  nerve  or  tract,  and  frequently 
bilateral,  is  found  in  disseminated  sclerosis.  It  does  not 
present  the  greyish  whiteness  of  the  tabetic  atrophy,  but 
in  cases  of  old  standing  may  result  in  complete  blindness. 
In  the  early  stages  of  the  disease,  temporary  attacks  of 
amblyopia  may  occur  without  any  obvious  ophthalmoscopic 
signs,  but  in  these  cases  some  degree  of  optic  atrophy  usually 
develops  in  the  later  stages. 

Secondary  atrophy.  Should  optic  neuritis  not  resolve 
under  appropriate  treatment,  the  natural  course  of  events  is 
towards  destruction  of  the  nerve  fibres,  atrophy  of  the  optic 
disc,  and  impairment  or  loss  of  vision.  In  this  condition  the 
disc  is  white,  the  margins  present  a  blurred  appearance,  the 
arteries  are  much  reduced  in  size,  and  white  lines  are  seen 
along  the  retinal  blood-vessels. 

Embolism  or  thrombosis  of  the  central  artery  of  the 
retina.  This  may  result  from  a  general  infective  malady, 
such  as  ulcerative  endocarditis,  or  from  local  arterial  disease. 
Thrombosis  of  the  central  artery  on  one  side  has  been 
observed  along  with  hemiplegia  of  the  opposite  side  of  the 
body.  It  is  a  rare  association,  resulting  from  extension  of 
thrombosis  from  the  internal  carotid  into  the  ophthalmic 
artery.  In  consequence  of  embolism  or  thrombosis  of  the 
central  artery,  anaemia  of  the  retina,  oedema,  hemorrhages 
and  atrophy  of  the  optic  disc  are  found. 

The  symptoms  are  sudden  impairment,  or  loss  of  vision 
from  which  a  recovery  may  to  a  large  extent  take  place. 

Optic  chiasma 

The  chiasma  may  be  involved  by  tumours  or  syphilitic 
growths  in  the  pituitary  fossa,  by  the  pressure  of  a  dilated 
third  ventricle,  as  in  hydrocephalus  and  in  some  cases  of 
acromegaly.  It  may  also  be  affected  by  local  lesions,  as  in 
disseminated  sclerosis  or  from  the  pressure  of  atheromatous 
arteries. 

The  symptoms  of  lesion  of  the  mesial  part  of  the  chiasma 
are  blindness  of  the  nasal  halves  of  the  retina,  with  loss  of 


THE  SPECIAL  .SENSES  01 

the  temporal  fields  on  both  sides  (bitemporal  hemi-anopsia). 
Lesions  of  the  external  surface  only  cause  blindness  of  the 
temporal  half  of  the  retina  (nasal  hemi-anopsia).     (Fig.  8.) 

Optic  tract 

Lesion  of  the  optic  tract  gives  rise  to  homonymous 
hemi-anopsia  to  the  opposite  side. 

The  chief  point  by  which  a  lesion  of  the  optic  tract  may 
be  distinguished  from  one  affecting  the  optic  radiations  is 
that  the  pupil  does  not  contract  when  light  is  thrown  on  the 
blind  halves  of  the  retina. 

HEARING  AND  EQUILIBRATION 

The  eighth  nerve  trunk  is  composed  of  two  separate  nerves 
— the  vestibular  nerve  from  the  semicircular  canals,  and  the 
auditory  proper  or  cochlear  nerve.  These  two  divisions  have 
separate  origins  and  terminations  and  different  functions. 

I.  The  vestibular  nerve 

Anatomy.  The  fibres  of  this  nerve  arise  in  the  bipolar 
cells  of  the  ganglion  vestibulare,  whose  peripheral  processes 
pass  to  the  lining  membrane  of  the  semicircular  canals.  The 
central  prolongations,  or  axones  combine  to  form  the  anterior, 
mesial  or  vestibular  root  of  the  eighth  nerve,  which  enters  the 
pons  Varolii  between  the  restiform  body  and  the  ascending 
trigeminal  root.  The  pontine  nuclei,  to  which  these  fibres 
pass,  are  not  exactly  known,  but  vestibular  root-fibres  may  be 
traced,  after  section  of  the  eighth  nerve  distal  to  the  accessory 
auditory  ganglion,  into  the  mesial  vestibular  nucleus,  the 
nucleus  of  Deiters  and  the  dorsal  vestibular  nucleus  of 
Bechterew.  Some  nerve  fibres  also  appear  to  come  into 
direct  relation  with  the  roof  nuclei  of  the  middle  lobe,  and 
perhaps  with  the  cortex  of  the  lateral  lobe  of  the  cerebellum 
(Cajal).  A  direct  connexion  between  the  vestibular  nerve 
and  the  nucleus  of  the  sixth  cranial  nerve  has  also  been 
observed.     (Fig.  10.) 

The  main  projection  system  from  these  nuclei  is  an 
indirect  one  to  the  cerebellar  cortex  of  the  middle  and 
lateral  lobes.  There  is  a  subsidiary  system  by  way  of  the 
internal  arcuate  fibres  of  the  pons. 


62 


NERVOUS  DISEASES 


Physiology.  The  vestibular  nerve  is  the  path  by  which 
impressions  from  the  semicircular  canals  reach  the  brain,  and 
more  especially  the  cerebellum.  Lesions  of  the  semicircular 
canals  in  animals  are  followed  by  profound  disturbances  of 
equilibration.  It  has  also  been  experimentally  proved  that 
section  of  the  vestibular  nerve  results  in  similar  well-marked 


:ORPORA  QUADRICEMINA 


Fig.  10  (from  Cunningham's  '  Anatomy  ' ). — Showing  the  central  connexions  of 
the  cochlear  and  vestibular  divisions  of  the  eighth  nerve. 


disturbances.  After  experimental  section  of  this  nerve,  the 
animal  rolls  many  times  in  succession  towards  the  side  of  the 
lesion.  Apparently  with  a  view  to  counteract  this  vertiginous 
condition,  the  limbs  upon  the  side  of  the  divided  nerve  are 
extended  and  abducted  from  the  body,  and  those  on  the 
opposite   side  are    flexed  and  adducted.      These  effects  are, 


THE  SPECIAL  SENSES  63 

however,  of  a  temporary  character,  the  animal  eventually 
regaining  its  equilibrium,  although  never  completely. 

Similarly  in  man,  lesions  of  the  labyrinth  and  of  the 
vestibular  nerve  are  associated  with  vertiginous  attacks  and 
disturbance  of  equilibration. 

According  to  Sherrington,1  the  labyrinth  is  '  the  receptor 
organ  '  of  the  head  segments,  which,  in  association  with  other 
receptive  organs  of  the  limbs  and  trunk,  form  a  proprio-ceptive 
system  for  the  maintenance  of  the  equilibrium,  the  head 
ganglion  of  which  system  is  the  cerebellum. 

2.  The  cochlear  nerve 

The  cells  of  origin  of  this  nerve  are  situated  in  the  gang- 
lion spirale.  They  are  of  a  bipolar  type,  the  peripheral 
processes  of  which  terminate  freely  between  the  epithelial  cells 
of  the  organ  of  Corti.  The  central  processes  form  the  posterior, 
lateral,  or  cochlear  root  of  the  eighth  nerve.  Many  of  the 
fibres  terminate  in  the  accessory  auditory  nucleus  or  auditory 
ganglion,  which  is  situated  on  the  trunk  of  the  nerve  outside 
the  pons,  while  the  remainder  pass  dorso-externally  over  the 
restiform  body  to  terminate  in  the  so-called  tuber culum 
acusticum,  which  lies  on  the  dorso-lateral  surface  of  the 
medulla.  It  is  probable  that  the  auditory  ganglion  and  the 
acoustic  tubercle  are  parts  of  the  same  structure,  to  which 
the  term  '  cochlear  end-nucleus '  has  been  applied,  as  section 
of  the  auditory  nerve  distal  to  this  structure  is  not  followed 
by  degeneration  of  the  central  conducting  fibres.  To  obtain 
degeneration  of  these  fibres,  the  cochlear  nerve  requires  to  be 
severed  proximal  to  the  auditory  ganglion,  and  by  this  means 
the  central  auditory  tract  may  be  in  part  demonstrated. 

The  central  auditory  tract  is  formed  of  the  fibres  of  the 
corpus  trapezoideum,  and  includes  the  superior  olivary  bodies 
and  both  lateral  fillets,  but  more  particularly  that  of  the  crossed 
side.  The  tract  is  continued  proximally  within  the  tegmentum 
cruris  as  far  as  the  internal  geniculate  body.  The  posterior 
quadrigeminal  bodies  would  appear  to  be  ganglia  accessory  to, 
rather  than  of,  the  central  auditory  tract.  From  the  internal 
geniculate  body  fibres  pass  to  the  temporal  lobe  by  way  of 
the  retro-lenticular  portion  of  the  internal  capsule  (figs.  7 
and  10),  and  its  destruction  has  been  stated  to  cause  deafness. 

1  Sherrington,  Brain,  1900. 


64 


NERVOUS  DISEASES 


There  is  a  complementary  corticifugal  tract,  which  passes 
from  the  first  temporal  gyrus,  through  the  centrum  ovale, 
and  the  lateral  pontine  s}'stem  of  the  crus,  to  terminate  in 
the  upper  portion  of  the  pons — the  temporo-pontine  tract. 
(Fig.  7.) 

Each  ear  stands  in  functional  relation  with  both  superior 
temporal  gyri,  as  a  unilateral  lesion  of  the  cortical  auditory 
centre  does  not  destroy  hearing  in  the  opposite  ear  alone,  hut 
probably  impairs  it  on  both  sides.  This  is  not  surprising  in 
view  of  the  existence  of  extensive  commissural  connexions 
between  the  two  auditory  tracts,  both  in  the  trapezoid  body, 
the  quadrigeminal  region,  and  the  internal  geniculate  bodies. 


Fig.  11  (after  Campbell). — This  figure  shows  the  cortical  representation  on 
the  convexity  of  the  centres  for  hearing  and  vision.  Temporal  lobe  : 
A,  primary  auditory  centre  ;  B,  audito-psychical  area.  Occipital  lobe  : 
A,  primary  visual  centre ;    B,  visuo-psychical  area. 

The  cortical  auditory  centres.  It  has  long  been  held 
that  the  cortical  auditory  centre  lies  in  the  superior  temporal 
convolution,  but  the  researches  of  Campbell  would  seem  to 
place  the  primary  auditory  centre,  or  centre  for  the  reception 
of  simple  auditory  impressions,  in  the  transverse  temporal 
gyri  of  Heschl,  which  occupy  the  posterior  end  of  the  superior 
surface  of  the  first  temporal  gyrus  within  the  Sylvian 
fissure.  These  are  hidden  from  view  until  the  lips  of  the 
Sylvian  fissure  are  separated.  As  in  the  case  of  the  other 
cortical  sensory  areas,  the  primary  auditory,  or  audito-sensory 
centre,  is  partly  surrounded  by  an  audito-psychical  centre, 
which   lies,    according    to    Campbell,    in    the    cortex    of   the 


THE  SPECIAL  SENSES  65 

convexity  of  the  hind  end  of  the  first  temporal  convolution.  In 
this  audito-psychical  centre — in  right-handed  persons,  on  the 
left  side  of  the  brain — the  auditory  speech-centre  is  situated. 
Lesion  of  this  area  is  associated  with  '  word- deafness,"  or 
the  loss  of  the  interpretation  of  spoken  language,  and  the 
power  of  recalling  words  and  names.     (Fig.  11.) 

The  auditory  cortical  centre  has  certain  subcortical 
connexions,  of  which  the  fasciculus  longitudinalis  inferior, 
connecting  it  with  the  primary  visual  centre,  and  the 
fasciculus  longitudinalis  superior,  connecting  it  with  the 
inferior  frontal  gyrus,  are  the  most  important. 

Diseases  or  the  Eighth  Nerve  and   its  Vestibular  System 

The  symptoms  of  paralysis  or  irritation  of  the  eighth  nerve 
consist  of  disturbances  both  of  the  sense  of  hearing  and  of 
equilibration. 

The  nerve  is  locally  affected  in  two  places :  (a)  in  the 
subdural  space ;  (b)  in  the  labyrinth  (cochlea  and  semicircular 
canals). 

Nerve  deafness 

1.  The  common  cause  of  nerve  deafness  is  lesion  of  the 
internal  ear  or  labyrinth.  This  may  either  be  a  primary 
disease  of  the  labyrinth,  to  which  the  term  Meniere's  disease 
has  been  applied,  or  it  may  be  secondary  to  an  old-standing 
suppurative  affection  or  sclerosis  of  the  middle  ear,  when 
the  term  '  Meniere's  symptom-complex '  is  applied  to  the 
condition. 

2.  Nerve  deafness  may  arise  from  paralysis  of  the 
auditory  nerve  at  the  base  of  the  brain.  This  is  commonly 
due  to  a  tumour  growing  either  from  the  sheath  of  the  nerve, 
or  from  the  periosteum  around  the  internal  auditory  meatus, 
involving  the  nerve.  The  deafness  in  these  cases  is  an  early 
symptom  and  may  persist,  with  slight  tinnitus  and  sometimes 
vertiginous  attacks,  for  a  considerable  period  before  the  onset 
of  other  symptoms  signifying  pressure  upon  adjacent  structures. 
These  symptoms  are  an  incomplete  degree  of  peripheral  facial 
paralysis,  unilateral  cerebellar  symptoms,  and  occasionally  an 
associated  anaesthesia  over  the  distribution  of  the  trigeminal 
nerve  (see  p.  267). 

5 


66  NERVOUS  DISEASES 

;>.  Nerve  deafness  may  arise  from  cortical  and  sub- 
cortical lesions.  In  cases  of  this  nature  the  lesion  is  usually 
softening  or  new  growth,  involving  the  temporal  lobe  and  more 
especially  the  first  temporal  gyrus.  A  few  cases  are  on  record 
in  which  deafness  has  been  due  to  softening  of  the  first 
temporal  gyrus  on  both  sides.  In  unilateral  lesions,  involving 
the  temporal  lobe  upon  the  left  side,  word-deafness  is  the  more 
common  symptom,  and  this  may  or  may  not  be  accompanied 
by  impaired  hearing  to  ordinary  auditory  impressions. 

The  absence  of  any  marked  degree  of  deafness  in  uni- 
lateral cortical  lesions  is  due  to  the  extensive  bilateral 
distribution  of  the  auditory  fibres  in  the  cerebral  cortex. 

Acute  labyrinthitis — Meniere's  disease 

Meniere's  disease  is,  in  its  true  form,  an  acute  destruction 
of  the  labyrinth,  arising  either  from  inflammation  (acute 
labyrinthitis)  or  from  hemorrhage  into  the  labyrinth  in  the 
course  of  arterio-sclerosis  or  syphilis. 

In  consequence  of  this  destruction  characteristic  symptoms 
are  found.  These  are  intense  vertigo,  vomiting,  a  feeling  as 
of  rotation  of  the  body  round  its  axis,  profound  disturbance 
of  equilibration,  and  a  well-marked  nystagmus  towards  the 
unaffected  side.  The  patient  is  of  necessity  confined  to  bed  and 
assumes  a  characteristic  attitude,  lying  upon  the  sound  side. 

The  severe  symptoms  last  from  two  to  three  days,  and  then 
gradually  subside ;  but  nystagmus  may  persist  for  a  longer 
period,  and  pronounced  movements  of  the  head  may  induce  a 
feeling  of  vertigo.  Eventually  all  the  irritative  symptoms 
pass  away,  leaving  only  unilateral  deafness  and  tinnitus 
aurium.  Acute  Meniere's  disease,  as  above  described,  is  a 
rare  malady. 

Chronic  labyrinthitis — auditory  vertigo— 
'  Meniere's  symptom-complex ' 

This  is  either  a  primary  condition  or  secondary  to  disease 
of  the  middle  ear. 

1.  The  primary  disease  occurs  mainly  during  early  adult 
and  adult  life.  It  is  often  of  a  progressive  character,  and 
may  be  ascribed  to  constitutional  causes — such  as  gout, 
senile  degeneration,  and  arterio-sclerosis. 


THE  SPECIAL  SENSES  67 

Symptoms.  The  symptoms  of  this  variety  show  all  degrees 
of  severity,  from  attacks  of  severe  vertigo,  impelling  the  patient 
forcibly  on  to  the  ground  and  followed  by  vomiting,  to  slight 
vertiginous  sensations,  which  may  or  may  not  be  succeeded 
by  nausea.  In  the  majority  of  cases,  more  or  less  '  nerve 
deafness  '  and  some  form  of  subjective  tinnitus  are  invariably 
present.  As  a  rule,  little  difficulty  is  experienced  in  the 
diagnosis  of  the  severe  type  of  the  malady  ;  but  in  cases  in 
which  the  aural  symptoms  are  only  slightly  developed,  the 
distinction  between  attacks  of  minor  labyrinthrine  vertigo 
and  minor  epileptic  seizures  is  not  easy.  The  presence, 
however,  of  labyrinthine  symptoms  favours  the  diagnosis  of 
the  local  condition. 

The  symptoms  are  either  unilateral  or  bilateral,  with  one 
ear  more  affected  than  the  other.  The  deafness  may  be  so 
slight  as  not  to  have  been  recognised  by  the  patient,  and  the 
tinnitus  is  of  a  hissing,  pulsating,  or  humming  character. 
The  attacks  of  vertigo  have  usually  been  preceded  for  some 
months,  or  a  year  or  more,  by  tinnitus  and  deafness. 

The  disease  is  not  necessarily  progressive,  although  symp- 
toms may  persist  for  long  periods.  Suitable  treatment  may 
keep  the  vertiginous  attacks  in  abeyance,  and  recovery 
eventually  ensue. 

2.  Deafness,  tinnitus,  and  vertigo  form  a  frequent  combina- 
tion in  those  who  have  previously  suffered  from  old-standing 
middle  ear  disease,  either  suppurative  or  sclerotic. 

The  symptoms  are  of  gradual  onset :  the  first  attack  of 
giddiness  having  been  preceded  for  an  indefinite  period  by 
tinnitus  and  deafness,  having  the  features  of  middle  ear 
deafness. 

Tinnitus  aurium  is  probably  the  most  distressing  symptom, 
owing  to  the  persistence  and  severity  of  the  sounds.  In  some 
cases  it  is  so  severe  as  to  be  actually  painful.  The  sounds 
vary  enormously — hissing,  knocking,  pulsating,  musical,  as  of 
a  train  in  a  tunnel,  humming,  &c. 

Vertigo  occurs  either  in  the  form  of  seizures,  in  which  the 
patient  is  hurled  suddenly  to  the  ground,  or  of  a  sensation 
as  if  he  has  been  struck  on  the  head.  The  sensation  may  be 
either  that  of  turning  to  one  or  other  side,  or  of  external 
objects  moving  in  one  or  other  direction.  Frequently  com- 
bined with  occasional  vertiginous  paroxysms  is  some  degree 


68  NERVOUS  DISEASES 

of  instability  in  walking,  so  that  the  patient  may  stagger  like 
a  drunken  man. 

In  old- standing  cases,  head  sensations  are  a  common  and 
persistent  symptom.  They  are  a  feeling  of  oppression  on 
the  vertex,  a  sensation  of  fullness  or  of  throbbing,  and 
headache. 

Diagnosis.  The  Meniere  group  of  symptoms  presents 
little  difficulty  in  diagnosis,  when  the  labyrinthine  deafness 
and  tinnitus  are  associated  with  severe  attacks  of  vertigo. 
Should,  however,  minor  and  temporary  vertiginous  seizures 
be  present,  without  subsequent  vomiting  or  nausea,  the 
deafness  and  tinnitus  being  partial  or  slight,  it  may  not  be 
easy  to  differentiate  the  condition  from  minor  epilepsy. 
In  epilepsy  some  interference  with  consciousness  is  always 
present.  There  would,  however,  appear  to  be  some  cases  in 
which  the  labyrinthine  disease  is  associated  with  attacks  of 
minor  epilepsy,  and  others  in  which  some  interference  with 
consciousness  is  a  feature  of  the  labyrinthine  vertigo.  The 
distinction  between  the  two  disorders  can  only  be  made  by  a 
careful  study  of  the  associated  phenomena :  impairment  of 
memory  and  mental  changes  in  epilepsy,  progressive  deafness 
and  tinnitus  in  labj'rinthine  disease. 

In  cases  presenting  profound  unilateral  '  nerve-deafness ' 
with  tinnitus  and  occasionally  slight  vertiginous  attacks,  the 
possibility  of  the  existence  of  a  tumour  of  the  auditory  nerve 
should  be  kept  in  mind  (p.  267). 

Treatment.  Bromide  of  potassium  is  the  most  useful 
remedy  in  the  treatment  both  of  Meniere's  disease  and  of 
Meniere's  symptoms.  It  should  be  administered,  as  in  the 
treatment  of  epilepsy,  over  long  periods  of  time.  In  most 
cases  its  administration  reduces  the  number  and  severity 
of  the  attacks,  and  in  many  arrests  them  entirely.  Hydro- 
bromic  acid,  though  favoured  by  many  physicians,  has  not 
been  found  so  satisfactory  as  the  bromide  salts. 

If  a  specific  cause  is  at  work  in  any  particular  case,  it 
should  be  combated  either  by  the  iodides,  or  by  alkalies, 
colchicum,  or  mild  purgation. 

Quinine  and  salicylate  of  soda  have  been  recommended  in 
cases  where  the  bromides  are  of  little  use,  but  in  our  experience 
their  administration  has  been  invariably  attended  by  an 
aggravation  of  the  symptoms. 


THE  SPECIAL  SENSES  69 

Local  counter-irritation,  or  the  application  of  a  seton 
behind  the  ear,  may  be  applied  in  conjunction  with  medicinal 
treatment. 

In  severe  cases — more  especially  if  unilateral,  progressive, 
and  with  painful  tinnitus — operative  destruction  of  the  laby- 
rinth or  division  of  the  auditory  nerve  has  been  recently 
practised. 


Part   IV 
THE    CRANIAL   NERVES 

The  paralyses  of  the  cranial  nerves  are  of  importance  and 
interest  not  only  as  indications  of  local  disease,  but  as 
evidence  of  localising  value  in  intracranial  disease. 

THE   OCULO-MOTOR   NERVES 

These  are  the  third,  fourth,  and  sixth  cranial  nerves. 
They  are  purely  motor  in  function. 

Etiology.  (1)  Paralysis  of  the  third,  fourth,  and  sixth 
cranial  nerves  may  be  due  to  traumata  of  the  bones  of  the 
skull  involving  the  sphenoidal  fissure,  periostitis,  tumours, 
and  inflammatory  conditions  within  the  orbital  cavity. 

2.  The  nerves  may  be  involved  within  the  skull,  in  their 
course  between  the  brain  and  the  foramina  of  exit,  by  new 
growths  or  inflammatory  conditions,  of  which  the  commonest 
are  syphilis  and  tubercle.  They  may  also  be  affected  in 
consequence  of  a  general  increase  of  intracranial  pressure. 
The  long  intracranial  course  of  the  sixth  nerves  renders  them 
especially  liable  to  suffer  from  this  cause.  They  may  also 
be  paralysed  as  a  result  of  neuritis — rheumatic  and  perhaps 
other  forms. 

3.  Toxic  causes — such  as  diphtheria  and  ptomaine 
poisoning — may  involve  either  the  nerves  or  their  nuclei  of 
origin. 

4.  The  nerves  or  the  nuclei  may  be  implicated  within  the 
pons  and  crura  cerebri  by  new  growths,  localised  vascular 
lesions  (thrombotic  or  hemorrhagic)  ;  and  in  polio-encephalitis 
either  superior  affecting  the  grey  matter  of  the  aqueduct  of 
Sylvius  and  third  ventricle,  or  inferior  affecting  the  grey 
matter  of  the  fourth  ventricle  and  aqueduct  of  Sylvius.     The 


THE  CRANIAL  NERVES  71 

nuclei  may  also  be  involved  in  a  general  polio-myelitis  and 
encephalitis,  and  from  hemorrhage  into  the  third  and  fourth 
ventricles.  The  nuclei  may  be  affected  by  chronic  degenerative 
processes,  either  limited  to  the  nuclei  of  the  ocular  muscles, 
or  in  association  with  similar  changes  in  the  bulbar  nuclei. 

5.  Ocular  paralyses  may  occur  during  the  course  of  some 
chronic  nervous  disorders,  especially  tabes  dorsalis  and  dis- 
seminated sclerosis. 

6.  Ocular  affections,  limited  to  the  conjugate  movements 
of  the  eyes,  may  be  due  to  cortical  and  subcortical  paralysis 
or  irritation. 

7.  Ocular  paralyses  may  also  be  found  in  association  with 
migraine,  myasthenia  gravis,  and  hysteria. 

8.  Congenital  ocular  paralyses. 

Symptomatology.  The  symptoms  of  ocular  paralyses  are 
of  two  kinds  :  (a)  subjective,  or  those  complained  of  by  the 
patient,  and  (b)  objective,  or  those  recognised  by  the  physician. 

The  subjective  symptoms  are  :  — 

1.  Diplopia,  or  double  vision,  is  the  result  of  strabismus. 
In.  certain  positions  two  images  are  seen — the  true  and  the 
false  image;  the  latter  always  being  observed  by  the  paralysed 
eye.  The  two  images  ought  to  be  described  by  the  patient  as 
regards  their  inclination  to  and  degree  of  separation  from 
each  other,  the  direction  in  which  this  increases,  and  the 
elevation  of  the  false  image  above  or  below  the  true. 

The  inclination  of  the  false  image  depends  on  the  degree 
of  rotation  of  the  eyeballs ;  and  its  separation  from  the 
true  image  is  in  proportion  to  the  amount  of  paralysis 
present. 

Homonymous  diplopia  is  that  in  which  the  false  image 
is  seen  on  the  same  side  as  the  paralysed  eye.  It  is 
characteristic  of  paralysis  of  the  abductor  muscles  of  the 
globes — viz.,  the  obliques  and  the  external  rectus. 

Diplopia  is  said  to  be  crossed  when  the  false  image  is 
seen  on  the  side  of  the  sound  eye.  This  is  characteristic  of 
palsy  of  the  adductors — the  internal,  superior  and  inferior 
recti. 

If  the  paralysed  muscle  is  also  an  elevator  of  the  eyeball, 
the  false  image  is  higher  than  the  true ;  if  a  depressor,  it  is 
on  a  lower  plane  than  the  true. 

In   cases     of     divergent     strabismus,    therefore,    diplopia 


72 


NERVOUS  DISEASES 


is    crossed  ;    in  cases  of    convergent  strabismus,  diplopia  is 
homonymous. 

2.  Giddiness  and  erroneous  projection  are  only  apparent 
when  the  action  of  the  healthy  muscle  exceeds  that  of  the 
affected  muscle.     They  are  most  marked  in  recent  cases. 

A  vertiginous  condition  may  be  induced  when  the  patient 
looks  only  with  the  paralysed  eye,  owing  to  nystagmoid  jerk- 

ings  occasioned  by 
attempts  to  keep  the 
image  on  the  macula. 
The  patient  may  in 
consequence  receive 
the  impression  that 
he  himself  is  oscil- 
lating. 

The  objective  signs 
are  :  — 

1.  Strabismus,  or 
squint.  This  is  of 
two  kinds  : 

(a)  True  or  con- 
comitant strabismus. 

(b)  Paralytic  stra- 
bismus. 

According  to  the 
deviation  of  the  visual 
axes,  strabismus  is 
said  to  be  convergent, 
divergent,  oblique,  su- 
perior, or  inferior. 

2.  Deviation  of  the 
ocular  axes.  This  is 
of  tw7o  kinds  : 

Primary  deviation 
is  that  which  occurs  in  the  paralysed  eye  on  attempting 
to  fix  an  object  when  the  normal  eye  is  covered ;  secondary 
deviation  is  that  observed  in  the  sound  eye,  and  occurs  from 
overaction  of  the  corresponding  muscle  when  the  patient  is 
asked  to  fix  with  the  paralysed  eye,  the  sound  eye  being  covered. 

3.  Limitation  of  movement  of  the  eyeball  is  in  the  direc- 
tion of  the  paralysed  muscle. 


Fig.  12. — Paralysis  of  the  left  internal  rectus 
muscle,  showing  external  strabismus  of 
the  left  eyeball. 


THE  CRANIAL  NERVES  73 

4.  Abnormal  attitudes  of  the  head.  In  order  to  avoid  or 
minimise  double  vision,  the  head  is  held  in  positions  accord- 
ing to  the  paralysis  present.  In  palsy  of  the  adductors  of 
the  eyeball  the  head  is  directed  to  the  sound  side,  and  in 
palsy  of  the  abductors,  to  the  affected  side.  In  paralysis  of 
the  elevators  the  head  is  thrown  backwards,  and  in  that  of 
the  depressors,  forwards  and  downwards. 


Pig.  13. — Paralysis  of  the  right  superior  rectus  muscle,  showing 
the  absence  of  upward  movement  of  the  right  eyeball  on  looking 
upwards. 

Paralysis  of  individual  ocular  muscles 

The  investigation  of  paralysis  of  the  individual  ocular 
muscles  is  facilitated  by  considering  them  as  either  adductors 
or  abductors  of  the  eyeball. 

The  adductors  of  the  eyes  are  the  internal,  superior,  and 
inferior  recti  muscles,  supplied  by  the  third  nerve. 

Internal  rectus.  This  is  solely  an  adductor  muscle.  If 
the  muscle  on   the   right  side  is  paralysed  the  diplopia  is 


74 


NERVOUS  DISEASES 


crossed,  separation  of  the  images  occurs  on  looking  to 
the  left,  the  images  are  parallel  and  on  the  same  level. 
(Fig.  12.) 

Superior  rectus.  This  is  an  elevator  and  also  an  adductor 
muscle.  In  paralysis  the  diplopia  is  crossed,  on  looking 
upwards  the  false  image  is  higher  than  the  true,  and  its 
separation  from  the  true  is  greater  above.  The  head  is  held 
backwards  and  inclined  to  the  healthy  side.    (Fig.  13.) 


Fig.  14. 


-Paralysis    of  the   right    external  rectus   muscle,    showing 
internal  strabismus  of  the  right  eveball. 


Inferior  rectus.  This  is  an  adductor  and  depressor  muscle. 
In  paralysis  the  diplopia  is  crossed  on  looking  downwards, 
and  the  separation  of  the  images  is  greater  below  the  hori- 
zontal.    The  head  is  slightly  flexed  towards  the  healthy  side. 

The  abductors  of  the  eyes  are  the  external  recti  and  the 
superior  and  inferior  oblique  muscles,  supplied  respectively  by 
the  sixth,  fourth,  and  third  nerves. 

External  rectus.  This  is  purely  an  abductor  muscle. 
The  diplopia  is  homonymous,  the  images  are  parallel  and  on 
the  same  level.     The  separation  of  the  images  increases  on 


THE  CRANIAL  NERVES 


75 


looking  to  the  paralysed  side.     The  face  is  turned  towards  the 
sound  side.     The  eye  is  deviated  inwards.     (Fig.  14.) 

Superior  oblique.  This  muscle  is  both  an  abductor  and  a 
depressor  of  the  globes.  It  moves  the  cornea  downwards  and 
outwards.  The  diplopia  is  found  on  looking  downwards,  and 
is  homonymous  ;  the  false  image  is  lower  than  the  true,  and  the 
greater  separation  is  at  the  lower  ends.  On  looking  downwards 
and  inwards  the  difference  in  the  height  of  the  images 
increases  ;  on  looking  downwards  and  outwards  the  obliquity 
increases.      The   head   is    turned  downwards   and   the  chin 


Left  SupV      />,      Right  Supr. 
rectus         !   \        rectus 


Left  fnfC 
rectus 


Right  Fixf' 
rectus 


Left  Infr 
obUaua 


/Right  LnfT. 
obliaue 


Left  Supr 
obliaue 


\  Right  Sup? 
\    obHaue> 


Fig.  15. — Werner's  '  Artificial  Memory '  for  the  position  of  the  false  images 
in  the  ocular  paralyses.  On  the  left  side  the  position  of  the  true  and 
false  images  is  shown  in  palsy  of  the  recti  muscles ;  on  the  right  side 
their  position  in  palsy  of  the  obliques.  The  continuous  line  represents 
the  true  image,  the  broken  line  the  false  image.  For  example,  in  paralysis 
of  the  right  superior  rectus,  the  false  image  is  crossed,  it  is  higher  than 
the  true,  it  is  most  marked  on  looking  upwards,  and  the  separation  of  the 
images  is  most  marked  above. 

inclined  towards  the  healthy  side.  Patients  with  this 
paralysis  complain  of  confused  vision  when  going  upstairs. 
Inferior  oblique.  It  is  an  elevator  and  an  abductor.  It 
carries  the  cornea  upwards  and  outwards.  The  diplopia  is 
homonymous,  is  seen  on  looking  upwards,  the  false  image 
is  higher  than  the  true,  and  the  greatest  separation  is  above. 
The  difference  in  the  vertical  separation  increases  on  looking 
upwards,  and  in  the  horizontal  on  looking  upwards  and 
outwards.  The  paralysis  of  movement  is  in  an  upward  and 
outward  direction ;  the  head  is  tilted  backwards  and  the  chin 
tilted  towards  the  sound  side. 


76 


NERVOUS  DISEASES 


Paralysis  of  individual  ocular  nerves 

In  complete  paralysis  of  the  third  nerve  there  is  palsy  of 
all  the  external  ocular  muscles  (except  the  superior  oblique 
and  the  external  rectus),  the  levator  palpebral  superioris,  the 
sphincter  pupillse,  and  the  ciliary  muscle.  The  eye  cannot 
be  rotated  upwards,  or  directly  inwards  or  downwards,  but  a 

slight  downward  and  in- 
ward movement  can  be 
effected  by  the  superior 
oblique.  The  eye  tends 
to  be  pulled  outwards 
(divergent  strabismus) 
by  the  unopposed  action 
of  the  external  rectus 
muscle.  The  pupil  is  at 
first  of  medium  size,  but 
later  dilates  owing  to 
the  unopposed  action 
of  the  dilator  pupillse. 
The  light  reaction  and 
the  power  of  accommo- 
dation are  lost.  If  the 
paralysis  is  complete, 
there  is  no  diplopia 
owing  to  the  ptosis  or 
drooping  of  the  upper 
eyelid.  To  counteract  the  ptosis  there  is  an  overaction 
of  the  corresponding  frontalis  muscle.     (Figs.  16  and  17.) 

In  partial  paralysis  any  one  of  the  individual  muscles 
supplied  by  the  nerve  may  be  affected.  As  a  general  rule 
there  is  some  degree  of  ptosis  and  some  interference  with  the 
pupillary  reactions. 

The  symptoms  of  paralysis  of  the  fourth  nerve  are  those 
already  described  for  the  superior  oblique  muscle. 

Paralysis  of  the  sixth  nerve  presents  the  symptoms 
already  described  under  palsy  of  the  external  rectus  muscle. 

Recurring-  ocular  paralysis 
This  is  a  rare  form  of  ocular   palsy,  in  which,  after  an 
attack  of  severe  headache  accompanied  by  vomiting,  paralysis 
of  an  ocular  nerve  is  temporarily  induced. 


Fig.  16.— Paralysis  of  the  left  third 
nerve,  showing  complete  ptosis  of 
the  left  upper  lid. 


THE  CRANIAL  NERVES 


77 


The    paralysis   is    usually 
sixth    may    also   be   affected, 
involves  the  levator  palpebral 
(Fig.  18.) 

In  typical  examples  of  this 
are  succeeded  by  oculo- 
motor paralysis  of  vari- 
able duration,  from  a 
few  days  up  to  several 
weeks.  As  the  malady 
progresses,  the  duration 
of  the  paralysis  is  pro- 
longed until  no  recovery 
takes  place,  and  the 
periodic  headaches  make 
no  alteration  in  the 
degree  of  the  palsy. 

In  all  cases  of  this 
character,  submitted  to 
post-mortem  examina- 
tion, an  organic  lesion — 
tumour  or  exudation — 
has  been  detected  involv- 
ing the  trunk  of  the 
affected  nerve. 


of  the    third    nerve,    but    the 

Occasionally    the    palsy  only 

or  the  internal  ocular  muscles. 

affection,  recurring  headaches 


Fig 


17. — A  case  of  partial  bilateral  pa- 
ralysis of  the  third  nerve.  Note  the 
double  divergent  strabisnms,  partial 
ptosis,  and  overaction  of  the  frontalis 

muscles. 


The    pupillary   light-reflex 

The  afferent  fibres  for  the  pupillary  light-reflex  are 
probably  distinct  from  the  visual  fibres,  and  pass  along  the 
optic  nerve,  through  the  chiasma,  into  the  optic  tract 
(p.  54). 

From  the  optic  tract  the  pupillary  fibres  enter  the  quad- 
rigeminal  region,  and  thence  are  conducted  by  way  of  Meynert's 
'fountain  decussation'  to  the  third  nerve  nucleus  (Harris).1 
From  this  locality  efferent  fibres  are  transmitted  through  the 
ciliary  nerves  to  the  ciliary  ganglion,  and  thence  to  the 
sphincter  iridis  muscle.  It  has  recently  been  shown  that 
the  mechanism   presiding  over  the  sphincter  action  of  the 


1  Harris,  Brain,  1904, 


78 


XER  VOL'S  DISEASES 


iris  lies  in  the  ciliary  ganglion  and  not  in    the  oculo-motor 
nucleus  (Bach).1 

The  pupil-dilating  fibres  pass  downwards  through  the  pons, 
medulla  oblongata,  and  cervical  portion   of  the  spinal  cord, 

probably  in  the  gela- 
tinous substance  or  ad- 
jacent posterior  horn. 
They  pass  from  the 
cord  through  the  an- 
terior roots  of  the  first 
and  second  dorsal 
nerves  and  enter  the 
inferior  cervical  gan- 
glion of  the  cervical 
sympathetic,  and  are 
transmitted  by  the 
sensory  root  of  the  fifth 
nerve,  the  ophthalmic 
branch,  and  the  long 
ciliary  nerves  to  the 
iris  muscle.  They  do 
not  pass  into  the  ciliaiy 
ganglion. 

Loss  of  the  light- 
reaction,  with  retention 
of  pupillary  contrac- 
tion on  convergence 
and  accommodation,  is  known  as  the  Argyll-Robertson  reaction. 
The  observations  of  Marina2  show  that  this  reaction  would 
appear  to  be  due  to  degenerative  changes  in  the  ciliary 
ganglion.  In  lesion  of  the  roots  of  the  third  nerve,  or  of 
the  nerve  trunk  itself,  the  pupil  is  dilated  and  inactive  on 
convergence.  Although  usually  a  bilateral  condition,  it  may 
be  found  on  one  side  only. 

The  converse  of  the  Argyll-Robertson  phenomenon  is 
occasionally  seen — viz.,  retention  of  the  pupillary  light-re- 
action with  loss  of  contraction  on  convergence. 


Fig.  18. — A  case  of  recurring  paralysis  af- 
fecting the  nerve  to  the  left  internal 
rectus  muscle. 


1  Bach,  Zeitsch.  fur  AugcnJieilkunde,  1904. 
-  Marina,  Annal.  di  Nercologia,  1901. 


THE  CRANIAL  NERVES  79 


Conjugate  movements  of  the  eyes 

Lateral  conjugate  movements  of  the  globes  are  produced 
by  the  associated  action  of  the  internal  rectus  muscle  of  one 
side,  and  the  external  rectus  of  the  other.  Both  muscles,  for 
the  purposes  of  this  movement,  are  innervated  from  the 
nucleus  of  the  sixth  nerve,  connecting  fibres  passing  from  this 
nucleus  to  the  opposite  third  nucleus.  Interference  with  this 
movement  may  therefore  be  due  to  lesion  (a)  of  the  fibres  which 
connect  the  cortical  centre  in  the  frontal  lobe  with  the  sixth 
nucleus,  (b)  of  the  sixth  nucleus,  or  (c)  the  commissural  fibres 
between  it  and  the  third  nucleus. 

A  unilateral  cerebral  lesion,  if  irritative,  causes  conjugate 
deviation  of  the  eyes  to  the  opposite  side ;  if  destructive  there 
is  paralysis  of  conjugate  movement  to  the  opposite  side,  and 
there  may  be,  if  the  lesion  is  recent,  conjugate  deviation  of 
the  eyes  to  the  same  side  as  the  lesion,  due  to  the  over- 
action  of  the  unaffected  cortical  centres  of  the  opposite 
hemisphere. 

Paralysis  of  conjugate  movement,  arising  from  a  lesion  in 
the  pons  Varolii,  involving  the  sixth  nucleus,  is  sometimes 
associated  with  facial  palsy  of  the  peripheral  type  upon  the 
same  side.  The  explanation  of  this  association  is  topographical, 
the  nucleus  of  the  sixth  nerve  being  surrounded  by  the 
intramedullary  root  of  the  seventh  nerve.  This  association  is 
absent  in  lesion  involving  the  commissural  fibres. 

The  other  conjugate  movements,  upward  and  downward,  are 
innervated  from  the  third  nerve  nucleus  and  are  rarely 
paralysed  alone,  although  defect  of  the  upward  movements  is 
found  as  a  symptom  of  lesion  in  the  neighbourhood  of  the 
quadrigeminal  bodies. 


Nystagmus 

Nystagmus  is  a  condition  in  which  on  conjugate  movements 
of  the  eyes,  the  ocular  deviation  is  poorly  sustained,  and  is 
effected  by  a  succession  of  slow  or  quick,  coarse  or  fine, 
rotatory  or  oscillatory  movements  of  varying  range. 

Nystagmus  may  arise  from  many  causes,  of  which  the 
following  are  the  chief :  — 


80  NERVOUS  DISEASES 

1.  Interference  with  the  co-ordinating  centres  and  con- 
nexions of  the  ocular  muscles — 

(a)  In  the  cerebellum.  Its  features  are  described  on 
p.  263. 

(6)  In  the  semicircular  canals.  Its  features  are  described 
on  p.  32. 

(c)  In  organic  diseases  of  the  midbrain  and  pons. 

(//)  As  a  temporary  symptom  in  some  cerebral  lesions. 

(c)  In  organic  diseases — such  as  disseminated  sclerosis  and 
Friedreich's  ataxy. 

(/)  In  paresis  of  the  ocular  muscles  in  peripheral  neuritis. 

2.  In  conditions  associated  with  interference  with  vision — 
such  as  optic  atrophy,  errors  of  refraction,  and  albinism. 

8.  From  strain  and  fatigue  of  the  ocular  muscles,  as  in 
high  myopia,  and  miner's  nystagmus. 

4.  As  a  congenital  condition  of  central  origin,  as  in  spasmus 
nutans. 

5.  Spontaneous  nystagmus.  In  this  the  movements  are 
irregular,  slow,  oscillating,  and  wandering.  They  may  be 
increased  on  conjugate  movement.  When  present  during 
rest,  they  are  due  to  an  inability  to  fix  the  eyes. 

THE  TRIGEMINAL  NERVE 

The  fifth  nerve  consists  of  two  primary  divisions — the  motor 
and  the  sensory — analogous  to  the  motor  and  sensory  roots  of 
the  spinal  nerves.  Situated  upon  the  sensory  division  is  the 
Gasserian  ganglion,  from  which  the  three  sensory  branches  of 
the  nerve  spring.  Its  deep  origin  and  connexions  have  been 
described  on  pp.  6  and  10. 

Sensory  distribution.  There  is  only  slight  overlap  between 
the  distribution  of  this  nerve  and  that  of  the  adjacent  cervical 
plexus.  The  sensory  loss  following  removal  of  the  Gasserian 
ganglion  is  considerably  less  than  the  anatomical  distribution 
of  the  nerve  upon  the  face.  The  areas  of  epicritic  and  proto- 
pathic  loss  are  almost  identical,  the  former  being  slightly  the 
larger  and  having  an  irregular  posterior  margin,  as  shown  in 
fig.  19  (Davies).  Excluded  from  the  area  of  trigeminal  dis- 
tribution is  notably  the  skin  of  the  external  auditory  meatus, 
the  auricle,  and  the  major  portion  of  the  lower  jaw.  The  area 
of  loss  of  deep  sensibility  probably  corresponds  to  that  of 


THE  CRANIAL  NERVES  81 

protopathic  anaesthesia.  The  trigeminal  distribution  over  the 
mucous  membranes  includes  the  nostril  and  half  of  the  tongue 
as  far  back  as  the  circumvallate  papillae,  the  margin  of  anaes- 
thesia passing  outwards  along  the  line  of  these  papillae  to  the 
anterior  pillar  of  the  fauces,  and  hence  along  the  centre  of  the 
soft  and  hard  palates  to  the  upper  lip  (Sherren).      The  fibres 


Fig.  19. — Chart  showing  the  area  of  anassthesia  following  excision  of  the 
Gasserian  ganglion.  The  deeply  shaded  portion  represents  the  area  of 
both  epicritic  and  protopathic  loss ;  the  lightly  shaded  shows  the  overlap 
of  the  epicritic  loss  (Davies). 

subserving  deep  sensibility  of  the  tongue  are  probably  conveyed 
in  the  hypoglossal  nerve. 

The  causes  of  trigeminal  paralysis  are  either  central  within 
the  pons,  or  peripheral,  affecting  the  nerve  roots.  Central 
paralysis,  both  motor  and  sensory,  may  result  from  tumours 
or  other  lesions  involving  the  central  connexions  of  the  nerves. 
The  motor  nucleus  may  be  the  seat  of  degenerative  changes 
in  bulbar  paralysis.  The  intramedullary  portion  of  the  sensory 
root  may  be  involved  in  tabes  and  syringomyelia. 

6 


82 


NERVOUS  DISEASES 


Peripheral  paralysis  is  usually  associated  with  pressure  upon 
the  roots  by  new  growths,  or  chronic  inflammatory  meningeal 
conditions. 

The  sensory  root  may  be  involved  alone  in  cases  of  herpes 
of  the  Gasserian  ganglion,  and  rarely  the  ganglion  itself  may 
be  destroyed  without  any  affection  of  the  motor  root. 

Motor  paralysis  is  limited  to  the  muscles  which  move 
the   lower  jaw.     Paralysis    with    atrophy    and   degenerative 


Fig.  20. — Paralysis  of  the  motor  division  of  the  left  fifth  nerve. 
The  lower  jaw  deviates  to  the  side  of  paralysis,  when  the 
mouth  is  opened. 

electrical  changes  are  observed  in  the  temporal  and  masseter 
muscles.  Paralysis  of  the  pterygoid  muscles'  is  shown  by  an 
inability  to  move  the  lower  jaw  to  the  healthy  side,  and  a 
deviation  of  the  jaw  to  the  paralysed  side  when  the  mouth  is 
opened.     (Fig.  20.) 

Sensory  paralysis.  Loss  or  impairment  of  the  epicritic, 
protopathic,  and  deep  sensibilities  is  found  over  the  cutaneous 
distribution    of    the   nerve.     The  mucous  membrane  of  the 


THE  CRANIAL  NERVES  83 

tongue  becomes  dry,  furred,  and  covered  with  the  debris  of 
food.  The  inside  of  the  cheek  may  show  ulcerated  patches, 
where  it  has  been  unintentionally  bitten  during  mastication. 
The  mucous  membrane  of  the  nose  is  dry  from  an  absence  of 
secretion,  and  the  sense  of  smell  is  impaired.  Trophic  ulcers 
may,  or  may  not,  develop  upon  the  cornea. 

The  pupil  on  the  side  of  the  lesion  is  usually  smaller  than 
the  normal  one.  Herpes  may  be  occasionally  observed  over 
the  distribution  of  the  fifth  nerve,  but  is  usually  confined  to 
the  supra-orbital,  nasal,  and  supra-trochlear  branches.  The 
cornea  is  also  sometimes  involved  in  this  condition. 

The  symptom  known  as  neuro-paralytic  keratitis  is  not  an 
essential  accompaniment  of  paralysis  of  the  sensory  root.  Its 
presence  would  seem  to  be  caused  by  irritation  of  the  sensory 
fibres  of  the  root,  or  of  the  Gasserian  ganglion.  In  surgical 
removal  of  the  ganglion,  it  may  be  prevented  by  closure  of  the 
lids  during,  and  for  a  few  days  subsequent  to,  the  operation, 
provided  always  that  no  septic  irritation  occurs  at  the  base  of 
the  brain.  We  hold  that  its  existence  is  evidence  of  a  neuritic 
or  inflammatory  affection  of  the  nerve  rather  than  of  paralysis. 

There  is  no  evidence  that  the  trigeminal  nerve  contains 
either  vaso-motor  or  trophic  fibres  for  the  skin  of  the  face,  nor 
is  there  any  alteration  in  lachrymal  or  salivary  secretion,  after 
section  of  the  nerve. 


THE   FACIAL   NERVE 

The  facial  is  a  mixed  nerve,  whose  motor  division  is  the 
portio  dura,  and  whose  sensory  division  is  the  pars  intermedia, 
or  nerve  of  Wrisberg,  which  takes  origin  in  the  ganglion 
geniculi  facialis. 

The  motor  division  supplies  all  the  facial  muscles  of 
expression,  the  stylo-hyoid  and  posterior  belly  of  the  digastric, 
the  platysma,  and  the  stapedius  muscles.  The  sensory  root  is 
distributed  to  a  skin  area  corresponding  to  the  anterior  surface 
of  the  external  ear  and  the  external  auditory  meatus.  This  is 
the  Zoster  area  affected  by  irritative  lesions  of  the  geniculate 
ganglion  (Ramsay  Hunt).  In  the  nerve  of  Wrisberg  are  also 
contained  taste  fibres  from  the  corda  tympani. 

The  whole  of  the  facial  nerve  enters  the  internal  auditory 
meatus  in  company  with    the    auditory   nerve.       Thence    it 


84  NERVOUS  DISEASES 

passes  through  the  aqueductus  Fallopii,  traverses  the  roof  of 
the  middle  ear  and  issues  from  the  temporal  hone  through  the 
stylo-mastoid  foramen.  On  the  face  it  is  divided  roughly  into 
three  main  hranches  :  the  oculo-facial,  supplying  the  frontalis, 
corrugator  supercilii,  and  the  orbicularis  palpebrarum  muscles, 
the  oro-facial  to  the  orbicularis  oris,  and  the  mid- facial  to  the 
remainder  of  the  muscles  of  expression. 

Facial  paralysis 

This  is  the  most  common  type  of  cranial  nerve  palsy,  and 
is  due  to  both  peripheral  and  central  causes. 

Etiology.  Of  peripheral  causation  the  most  frequent  is 
the  so-called  '  rheumatic,'  or  refrigeration  paralysis.  Eighty- 
eight  per  cent,  of  our  cases  arose  from  this  cause,  the  remainder 
being  due  to  suppurative  middle  ear  disease,  pressure  from 
the  forceps  at  birth,  or  in  consequence  of  operations  upon  the 
mastoid  bone.  Other  peripheral  causes  of  facial  palsy  are 
basal  syphilitic  meningitis  and  the  pressure  of  tumours  in 
the  cerebellar  pontine  angle. 

Peripheral  facial  paralyses  of  rheumatic  origin  have  a 
certain  seasonal  incidence.  In  our  series  the  months  of  April, 
October,  and  November  gave  the  greatest  frequency — the  cases 
amounting  to  nearly  one- half  of  the  total  number.  This 
would  suggest  an  infective  causation,  corresponding  in  many 
ways  to  the  seasonal  incidence  of  acute  poliomyelitis.  The 
occasional  occurrence  of  the  malady  in  families,  or  amongst 
those  who  are  working  in  the  same  locality,  also  supports 
this  view. 

Pathology.  The  pathological  change  underlying  this  form 
of  facial  palsy  is  a  parenchymatous  neuritis,  with  disintegra- 
tion of  the  medullary  sheaths.  It  is  most  apparent  in  the 
peripheral  distribution  of  the  nerve  and  at  the  distal  end  of 
the  Fallopian  canal,  with  a  lessening  intensity  as  the  nerve 
is  traced  towards  the  geniculate  ganglion. 

Facial  paralysis  is  favoured  by  a  congenital  narrowing  of 
the  Fallopian  canal  and  stylo-mastoid  foramen.  Gowers  has 
described  the  relative  frequency  of  peripheral  facial  palsy  in 
hemiatroplna  facialis,  in  which  the  facial  bones  are  atrophied. 

Symptoms.  In  the  common  type  of  facial  palsy,  the 
muscles  of  one  side  of  the  face  are  completely  paralysed.  The 
patient  is  unable  to  frown  or  raise  the  eyebrows,  to  close  the 


THE  CRANIAL  NERVES  85 

eyelids,  to  whistle,  or  to  smile.  The  naso-labial  fold  is 
obliterated,  and  in  old- standing  cases  the  angle  of  the  mouth 
may  hang  down  in  a  pouch-like  fashion.  All  the  wrinkles 
and  furrows  of  the  face  on  the  paralysed  side  are  erased,  in 


Fig.  21a. — A  case  of  old-standing,  right-sided,  peripheral  facial 
paralysis.  Note  the  inability  to  close  the  right  eyelids, 
the  pouch-like  appearance  of  the  right  side  of  the  face,  and 
the  absence  of  the  naso-labial  fold. 

consequence  of  which  it  assumes  an  expressionless,  mask-like 
appearance.     (Fig.  21a.) 

In  eating  and  drinking,  food  collects  in  the  paralysed 
cheek,  and  fluid  may  run  out  at  the  angle  of  the  mouth  ;  tears 
roll  down  the  face  from  weakness  of  the  lower  lid,  which  is 
sometimes  everted  into  a  condition  of  ectropion,  with  resulting 
conjunctivitis. 

The  tongue  may  present  a  false  appearance  of  being- 
protruded  to  the  healthy  side.     The  sensibility  of  the  face  is 


86  NERVOUS  DISEASES 

not   impaired,  though    pain — usually  behind   the  ear — is    a 
frequent  symptom  in  the  early  stages. 

The  sense  of  taste  may,  or  may  not,  be  abolished  on  the 
anterior  two-thirds  of  the  tongue  on  the  paralysed  side. 
Taste  may  not  be  affected  at  all  in  mild  cases  of  paralysis, 
owing  to  the  limitation  of  the  neuritis  to  the  nerve  distribution 


Fig.  21b. — Depicts  an  old  case  of   facial  palsy  of   the   left 
side  showing  paralysis  on  voluntary  movement. 

upon  the  face.  In  more  severe  cases,  on  the  other  hand, 
taste  is  usually  lost  early  in  the  disease,  owing  to  neuritic 
extension  along  the  bony  canal,  involving  the  corda  tympani. 

Hearing  is  often  implicated.  If  the  facial  neuritis  is 
secondary  to  suppurative  otitis  media,  air  conduction  is 
abolished,  and  the  Rinne  reaction  is  negative  ;  but  in  cases 
where  the  nerve  to  the  stapedius  is  paralysed,  an  increased 
sense  of  hearing  (hyperaceusis)  may  be  present,  especially  to 
musical  sounds. 

Tn    severe   forms  of  facial    palsy,  the    muscles    show  the 


THE  CRANIAL  NERVES 


87 


reaction  of  degeneration  in  from  a  week  to  ten  days  after  the 

onset.     In  milder  cases,  a  diminution  of  faradic  irritability 
only  is  detected. 

Peripheral  facial  palsy  is  usually  one-sided,  but  a  bilateral 

palsy  is  occasionally  observed.      The   two    sides  are  rarely 

simultaneously   affected,    and   one   side   is   more  completely 
paralysed  than  the  other.     (Pigs.  22  and  23.) 


Fig.  22. 


Fig.  23. 


A  case  of  bilateral  facial  paralysis. — Fig.  22,  when  at  rest ;  fig.  23,  on  attempt  at 
movement.  Observe  that  the  left  side  is  more  paralysed  than  the  right. 
The  mask-like  appearance  and  absence  of  expression  are  veil  shown. 

In  the  ordinary  mild  type  of  the  disease,  complete  recovery 
ought  to  occur.  But  a  number  of  cases  of  a  more  severe 
character  are  not  uncommon,  in  which  secondary  contracture 
ensues.  This  condition  is  often  accompanied  by  a  feeling  of 
stiffness  of  the  face,  and  in  states  of  repose,  the  contracture 
of  the  paralysed  muscles  may  induce  a  false  appearance  of 
weakness  on  the  healthy  side.  In  these  cases,  attempts  to 
close  the  eyelids  will  result  in  partial  failure  and  in  an  over- 
action  of  the  levator  anguli  oris,  so  that  the  mouth  will  be 
pulled  forcibly  over  to  the  paralysed  side ;  on  showing  the 
teeth  the  mouth  is  pulled  to  the  non-paralysed  side. 

Facial  palsy  may  coexist  with  herpes  zoster  of  the  auricle, 
external  auditory  meatus,  and  sometimes  of  the  superficial 
cervical  plexus,  in  whole  or  in  part.  In  severe  cases  of  this 
character  deafness  and  symptoms  of  labyrinthine  affection 


88  NERVOUS  DISEASES 

may  also  be  present.  This  '  syndrome  '  would  appear  to  he 
due  to  an  acute  infective  inflammation  of  the  geniculate 
ganglion  with  secondary  implication  of  the  adjacent  facial 
and  auditory  nerves  in  the  internal  auditory  meatus 
(Ramsay  Hunt).  (Fig.  24.) 
As  facial  paralysis  may  arise  from  destructive  lesion  any- 


Fig.  24. — A  rase  (if  ri^ht-skled  facial  palsy  with  herpes  of  the 
superficial  cervical  plexus.  The  herpetic  vesicles  have  been 
artificially  stained  black. 

where  between  the  cerebral  cortex  and  the  facial  musculature, 
several  clinical  types  are  observed. 

1.  A  cortical  and  subcortical  palsy,  in  which  the  oculo- 
facial muscles  are  relatively  less  involved  than  those  about 
the  angle  of  the  mouth.  The  upper  group  is,  however, 
impaired,  for  although  the  eyelids  may  be  closed  voluntarily, 
the  patient  may  be  unable  to  keep  them  closed  against 
resistance. 

In  bilateral  paresis — such  as  occurs  in  the  pseudo -bulbar 
palsies — there  is  usually  an  overaction  of  the  frontalis 
muscle,  giving  rise  to  a  characteristic  transverse  furrowing 
of    the    forehead,   and   an   associated   paresis  of  the  tongue 


THE  CRANIAL  NERVES  W) 

movements,  defective  articulation,  and  paralysis  of  the 
limbs.  The  electrical  irritability  of  the  muscles  is  un- 
changed. 

2.  Nuclear  paralysis  is  usually  of  the  chronic  type  and 
rarely  occurs  alone,  being  found  in  association  with  atrophic 
paralysis  of  the  bulbar  and  motor  trigeminal  nerves,  and 
with  atrophy  of  the  small  muscles  of  the  hands,  arms,  and 
shoulders.  The  lower  part  of  the  face,  more  particularly  the 
orbicularis  oris,  is  mainly  affected  and  contrasts  in  a 
striking  manner  with  the  over-action  of  the  frontalis.  In 
these  cases  electrical  alterations  are  of  a  quantitative  rather 
than  a  qualitative  character. 

3.  Boot  paralysis.  The  facial  nerve  root  is  not  in- 
frequently involved  by  tumours  of  the  tegment  of  the  pons 
and  of  the  posterior  fossa.  In  these  cases  the  palsy  is  of 
the  peripheral  type,  and  is  rarely  complete.  It  is  usually 
associated  with  palsy  of  the  adjacent  trigeminal  and  auditory 
nerves.  The  sense  of  taste  on  both  the  front  and  back  of  the 
tongue  on  the  paralysed  side  may  be  impaired  or  lost. 

4.  Facial  paralysis  of  a  peripheral  type  is  observed  in 
diphtheritic,  alcoholic,  and  lead  neuritis.  Weakness  of  the 
facial  muscles  is  also  seen  in  the  myopathies  and  myasthenia 
gravis. 

The  prognosis  of  the  refrigeration  paralysis  is  based  upon 
a  study  of  the  electrical  reactions.  If  from  a  week  to  ten 
days  after  the  onset  there  is  only  quantitative  diminution  of 
faradic  excitability  in  the  paralysed  muscles,  recovery  will 
take  place  in  from  three  to  four  weeks,  or  less.  Should  the 
faradic  reaction  be  abolished  with  qualitative  galvanic 
alterations,  recovery  may  be  indefinitely  delayed.  If  these 
reactions  persist  over  many  months,  ultimate  complete 
recovery  is  doubtful,  although  some  degree  of  return  of 
movement  is  probable  with  contracture  of  the  paralysed 
muscles. 

A  favourable  type  of  facial  palsy  is  that  arising  from 
temporary  pressure  upon  the  facial  nerve  accidentally  inflicted 
during  the  performance  of  the  complete  mastoid  operation. 

Treatment.  In  mild  cases  a  daily  application  of  the 
faradic  current  will  assist  the  natural  tendency  to  resolution 
and  recovery.  In  cases  in  which  the  faradic  reaction  is 
abolished,   the   application  of  the  continuous  current  for  a 


90  NERVOUS  DISEASES 

prolonged  period  is  essential.  Massage  of  the  facial  muscles 
in  all  cases  is  of  undoubted  value  in  tending  to  preserve  the 
muscular  nutrition  and  the  lines  and  contour  of  the  face, 
and  to  prevent  contracture  of  the  paralysed  muscles.  On 
the  return  of  some  faradic  excitability,  combined  galvano- 
faradism  may  be  usefully  adopted.  In  cases  arising  from 
otitis  media,  the  cure  of  the  aural  condition  is  essential,  and 
this  may,  if  necessary,  be  followed  by  an  anastomosis  of  the 
peripheral  end  of  the  cut  facial  nerve  with  the  hypoglossal 
nerve. 

Facial  spasm  is  described  under  The  Tics,  on  p.  588. 


THE  GLOSSOPHARYNGEAL  AND  VAGUS  NERVES 

The  nerves  distinguished  by  these  names  are  in  reality 
portions  of  one  large  mixed  nerve,  having  a  common  origin 
for  their  motor  and  a  common  termination  for  their  sensory 
fibres  in  the  medulla  oblongata.  The  nerve  roots,  as  they 
issue  from  and  enter  the  medulla,  form  a  series  of  fasciculi 
between  the  restiform  body  and  the  inferior  olive.  To  the 
uppermost  of  these  roots  the  term  'glossopharyngeal'  has  been 
applied,  and  to  the  middle  '  vagus.'  To  the  lowest  the  term 
'  accessory '  has  been  applied  under  a  misapprehension  :  they 
being  in  reality  the  most  distal  of  the  vagal  roots.  The  term 
'  accessory  '  was  originally  given  by  Willis  to  the  nerve  which 
springs  from  the  spinal  cord,  enters  the  skull  through  the 
foramen  magnum,  and  issues  therefrom  in  company  with  the 
vagi  through  the  jugular  foramen. 

The  deep  origin  and  connexions  have  been  described  on 
pp.  7  and  10. 

The  peripheral  distribution  of  the  vagoglossopharyngeal 
nerve  is  very  extensive,  embracing  such  structures  as  the  soft 
palate,  the  vocal  cordis,  the  tongue  (in  part),  the  pharynx  and 
oesophagus,  the  lungs,  heart,  stomach,  and  to  some  extent 
also  the  intestines. 

Etiology.  This  nerve  may  suffer  paralysis,  in  whole  or  in 
part,  from  lesions  of : — 

(a)  Its  medullary  nuclei :  as  in  bulbar  palsy,  tabes, 
syringomyelia,  and  disseminated  sclerosis. 

(6)  Its  intracranial  roots  :  as  in  lesions  of  the  meninges, 


THE  CRANIAL  NERVKS  91 

intracranial  tumours,  aneurisms,  and  disease  of  the  bones  of 
the  base  of  the  skull. 

(c)  Its  extracranial  course :  from  tumours,  injuries,  and 
other  sources  of  pressure.  The  vagus  nerve,  particularly  its 
recurrent  laryngeal  branch,  is  especially  prone  to  compression 
on  the  left  side  from  aneurism  of  the  arch  of  the  aorta,  and 
from  mediastinal  growths  and  tuberculous  disease  of  the  apex 
of  the  right  lung. 

(d)  Certain  poisons  and  general  infective  disorders  affect  the 
vagus  as  part  of  an  extensive  peripheral  neuritis.  Of  these 
may  be  mentioned  alcohol,  lead,  arsenic,  diphtheria,  and 
influenza. 

Symptoms.  The  symptoms  vary  according  to  the  position 
of  the  lesion  ;  but  may  be  separated,  for  descriptive  purposes, 
into  those  referred  to  (a)  the  glossopharyngeal  nerve,  and  (6) 
the  vagus  nerve. 

I.  Glossopharyngeal  paralysis 

There  is  no  case  on  record  of  isolated  palsy  of  this  nerve. 
The  trunk  of  the  nerve  contains  the  fibres  of  taste  for  the 
posterior  third  of  the  tongue,  but  it  is  uncertain  how  far  taste 
fibres  are  contained  in  the  roots.  Owing  to  a  branch  which  is 
given  off  from  the  petrous  ganglion,  the  nerve  gives  fibres  to 
the  tympanic  plexus  for  the  supply  of  the  mucous  membrane 
of  the  tympanum  and  the  Eustachian  tube.  The  pharyngeal 
branches  fuse  with  similar  branches  of  the  vagus  to  form 
the  pharyngeal  plexus,  which  is  both  motor  and  sensory  for 
the  superior  constrictor  muscle  and  mucous  membrane  of  the 
pharynx.  Symptoms  of  glossopharyngeal  palsy,  therefore, 
are  difficulty  in  swallowing,  anaesthesia  of  the  upper  pharynx, 
and  loss  of  taste  over  the  posterior  third  of  the  tongue. 

2.  Vagus  paralysis 

The  larynx  is  supplied  by  two  branches  of  the  vagus  nerve  ; 
the  superior  laryngeal,  which  is  sensory  for  the  mucous  mem- 
brane of  the  larynx  above  the  vocal  cords  and  motor  for  the 
cricothyroid  and  epiglottideus  muscles ;  and  the  recurrent 
laryngeal,  which  supplies  the  remaining  muscles  and  the 
mucous  membrane  below  the  vocal  cords  and  the  trachea. 


92 


NERVOUS  DISEASES 


The    character  and    symptoms    of   the    several    forms    of 
laryngeal  paralyses  are  indicated  in  the  following  table  : — 


Paralysis. 


Symptoms. 


Complete    bilateral    Aphonia,  stridor,  in 


palsy 


Complete  unilateral 
palsy 


ability  to  cough 


Bilateral    abductor 
palsy 


Unilateral  abductor 
palsy 


Voice  hoarse,  cough 
impossible,  stri- 
dor on  deep  in 
spiration 


State  of  Vocal  Conn. 


Motionless     in    the 
cadaveric       posi 
tion 


Oadses. 


Paralysed  and  mo 
tionless  in  cada- 
veric position 


Voice  not  affected, '  Cords    approximate 


inspiratory  stri- 
dor, cough  nor- 
mal 


Bilateral    adductor 
palsy 


Voice  and  cough  not 
affected.  May  be 
slight  inspiratory 
stridor 


Aphonia 


on  phonation.  Do 
not  separate  in 
inspiration 


Organic  only,  as 
in  tabes 


Organic,  usually 
pressure  on 
recurrent  lar- 
yngeal nerve 


Organic,  and  in- 
dicates early 
palsy 


Cords    approximate  Early      organic 
on  phonation.  Par-       disease 
alysed  cord,  immo- 
bileon  inspiration 


Inspiratory      move- :  Functional 
ment  normal.  Im-I 
perfect  movement 
on  attempted  pho- 
nation. 


In  the  earlier  stages  of  pressure  upon  the  vagus,  and  in 
nuclear  degeneration,  the  first  symptom  to  be  observed  is 
paralysis  of  the  abductor  mechanism  (Semon).  This  is  a 
well-recognised  clinical  phenomenon,  and  may  be  due  to  the 
fact  that  the  abductor  and  adductor  fibres  occupy  distinct 
and  separate  bundles  in  the  nerve. 

Other  symptoms  are  found  in  paralysis  of  the  soft  palate, 
either  on  one  or  both  sides,  according  to  the  position  of  the 
lesion  and  anaesthesia  of  the  laryngeal  mucous  membrane. 

In  bilateral  affections  of  the  vagi,  there  is  irregularity  in 
and  acceleration  of  the  heart's  action.  This  is  due  to 
paralysis  of  the  cardio-inhibitory  mechanism  derived  from 
the  upper  vagal  nerve  roots.  Symptoms  referred  to  the  oeso- 
phagus, stomach,  intestine,  and  lungs,  although  theoretically 
possible,  and  stated  to  exist  by  some  writers,  are  vague  and 
not  sufficiently  authenticated  to  be  regarded  as  characteristic. 


THE  CRANIAL  NERVES 


93 


THE  HYPOGLOSSAL  NERVE 

This  nerve  is  frequently  paralysed  in  cerebral  disorders. 
It  is  a  common  accompaniment  of  hemiplegia,  from  lesion  of 
the  supranuclear  fibres.  The  nucleus  is  involved  in  bulbar 
palsy,  and  may  be  in  tabes  and  syringomyelia.  The  nerve 
roots  are  affected  in  basal  meningeal  lesions  and  in  caries 
of  the  occipito-atloid  region.      In   the  neck  the  nerve  may 


Pig.  25. — A  case  of  syringo- 
bulbia, showing  para- 
lysis and  atrophy  of  the 
left  side  of  the  tongue. 


Fig.  26. — Bilateral  paralysis  of  the 
tongue  from  an  intramedullary 
lesion  affecting  the  roots  of  the 
hypoglossal  nerves. 


be   injured    by    wounds   or    by   the    pressure    of    tumours. 
(Figs.  25  and  26.) 

Symptoms.  In  supra-nuclear  paralysis  of  this  nerve  the 
tongue  is  protruded  towards  the  paralysed  side  in  a  curved 
fashion.  It  can  be  moved  from  side  to  side  within  the 
mouth,  but  there  may  be  some  interference  with  the  formation 
of  the  lingual  consonants.  If  the  lesion  is  nuclear,  or  infra- 
nuclear,  the  tongue  shows  marked  wasting  either  on  one  or 
both  sides  ;  the  mucous  membrane  is  wrinkled  and  fibrillary 
tremors  of  its  muscles  may  be  detected.     In  bilateral  palsy  it 


94  NERVOUS  DISEASES 

may  not  be  possible  to  protrude  it  beyond  the  dental  arch. 
In  these  cases  a  difficulty  in  chewing  and  swallowing  is 
usually  superadded.     (Fig.  26.) 


Fig.    27. — Unilateral   paralysis   of    the   tongue,  which   is 
protruded  to  the  left  side  or  the  side  of  paralysis. 


SPINAL    ACCESSORY   NERVE 

This  nerve  supplies  the  sterno-mastoid  and  the  upper  third 
of  the  trapezius  muscles. 

It  may  be  paralysed  in  consequence  of  disease  of  the  upper 
cervical  region  as  a  part  of  a  progressive  muscular  atrophy, 
and  in  caries  of  the  cervical  vertebrae,  especially  affecting 
the  occipito-atloid  and  atlo-axoid  joints. 

Its  paralysis  is  seen  in  impairment  of  the  normal  promi- 
nence and  outline  of  the  neck,  by  wasting  of  the  upper  part 
of  the  trapezius  and  by  drooping  of  the  shoulder.  Paralysis 
of  the  sterno-mastoid  alone  is  shown  by  an  inability  to  turn 
the  head  to  the  opposite  side,  while  on  depression  of  the  chin, 


THE  CRANIAL  NERVES 


95 


the  head  deviates  towards  the  sound  side.  Palsy  of  the 
trapezius  alone  is  demonstrated  by  an  inability  to  shrug 
the  shoulder. 

Under  this  heading  mention  may  be  made  of  a  permanent 
contraction   of  the   sterno-mastoid,  giving  rise  to  a  form  of 


Fig.  28. — Paralysis  of  the  right  spiual  accessory  nerve,  showino- 
the  absence  of  the  normal  outline  of  the  neck,  with  droopino- 
of  the  shoulder. 


'  wry  neck.'  This  condition  may  be  congenital  from  intrau- 
terine injury,  or  follow  upon  injury  to  the  spinal  accessory 
nerve  or  sterno-mastoid  muscle  during  delivery,  or  in  the 
case  of  adults  from  any  traumatism  about  the  neck.  The 
symptoms  are  :  an  inability  to  turn  the  head  towards  the  side 
of  the  paralysed  muscle,  and  a  fixation  of  the  head  in  the 
direction  of  the  normal  action  of  the  sterno-mastoid  muscle. 


Part  V 
THE  PERIPHERAL  NERVOUS  SYSTEM 

STRUCTURE   OF   THE    PERIPHERAL   NERVES 

A  cross-section  of  a  peripheral  mixed  nerve  shows  (a)  an 
epineurium  or  nerve  sheath ;  (b)  a  perineurium,  which  sur- 
rounds bundles  of  nerve  fibres  and  gives  off  (c)  an  endoneurium, 
which  surrounds  the  individual  nerve  fibres. 

The  nerve  sheath  contains  fat,  blood-vessels,  and  small 
nerves  (nervi  nervorum).  Small  blood-vessels  also  pass  with 
the  perineurium  to  supply  nerve  fibres.  In  the  peri-  and 
endoneurium  are  lymphatic  channels  which  communicate 
with  the  general  lymphatic  system. 

The  spinal  nerves  consist  mainly  of  medullated  fibres, 
but  some  probably  contain  non-medullated  fibres.  The 
medullated  fibres  are  composed  of  an  axis-cylinder,  a  medullary 
sheath,  and  a  neurilemma  sheath.  The  axis-cylinder  which 
lies  near  the  centre  of  the  fibre  is  formed  of  fine  fibrillar  The 
medullary  sheath  surrounds  the  axis-cylinder,  and  is  a  homo- 
geneous retractile  structure  composed  of  myeline.  When 
degenerating,  this  breaks  up  into  droplets,  which  stain  black 
with  osmic  acid.  The  myeline  sheath  is  surrounded  by  the 
neurilemma  sheath,  a  structureless  elastic  membrane.  At 
certain  places  the  myeline  is  absent  or  much  attenuated,  so 
that  the  axis-cylinder  appears  to  be  directly  surrounded  by 
the  neurilemma  sheath.  These  are  known  as  the  nodes  of 
Ranvier,  and  between  each  pair  of  nodes  is  the  neurilemma 
nucleus,  which  is  supposed  to  possess  a  nutritive  influence 
over  the  nerve  fibre.  In  addition  to  these  nodes  obliquely 
placed  incisures  of  the  myeline  sheath,  known  as  Lanter- 
mann's  incisures,  are  present. 

Non-medullated  or  grey  fibres  are  found  chiefly  in  the 
sympathetic  and  olfactory  nerves. 

96 


THE  PERIPHERAL  NERVOUS  SYSTEM  07 


Ohapteb   I 

LESIONS  OF  THE  PERIPHERAL  NERVHS 

Etiology.  Local  lesions  of  the  peripheral  nerves  may 
arise  from  the  following  causes  : — 

1.  Local  inflammatory  conditions — such  as  syphilis,  gout, 
and  rheumatism. 

2.  Wounds — incised,  lacerated,  gunshot,  and  operative. 
Accidental  injuries  are  specially  common  about  the  wrist,  while 
gunshot  injuries  are  more  frequent  in  the  sciatic  and  musculo- 
gpiral  nerves.  Injuries  to  nerves  may  also  be  inflicted  during 
an  operation.  The  facial  nerve,  the  cervical  plexus,  and  the 
spinal  accessory  nerve  are  especially  liable  to  injury  during 
operations  upon  the  ear  and  in  the  neck. 

3.  Stretching  and  tearing  of  the  nerve.  This  may  result 
from  excessive  traction — such  as  may  arise  from  traction 
exerted  during  delivery,  from  a  fall  upon  the  shoulder 
or  side  of  the  head,  from  manipulations  of  joints  under 
anaesthesia,  from  the  breaking  down  of  contractures,  or  from 
excessive  traction  upon  the  arm,  as  in  operations  upon  the 
breast  or  axilla. 

4.  Pressure.  Pressure  upon  nerves  may  be  due  to 
tumours,  dislocations,  fractures,  callus,  fibrous  adhesions 
around  or  in  the  neighbourhood  of  nerves,  and  to  the 
pressure  of  bandages,  splints,  and  crutches. 

Nerve  injuries  found  in  association  with  fractures  may 
be  primary,  and  due  either  to  direct  laceration  of  the  nerve 
by  a  fragment  or  splinter  of  bone  penetrating  the  nerve,  or 
to  the  actual  violence  which  leads  to  the  fracture  causing 
traction  on  or  contusion  of  the  nerve.  This  injury  may  occur 
at  some  distance  from  the  fracture. 

Secondary  lesions  of  nerves  may  occur  at  a  late  period 
when  the  nerve  sheath  is  irritated  or  involved  by  callus  or 
fibrous  tissue. 

Many  nerve  lesions  may,  without  causing  any  solution 
of  the  anatomical  continuity  of  the  nerve,  give  rise  to  a 
temporary  or  permanent  loss  of  function.  Physiologically,  the 
function  of  the  nerve  may  be  impaired  or  abolished;  anatomi- 
cally, the  nerve  may  be  completely  or  partially  severed.     It 

7 


98  NERVOUS  DISEASES 

is  obvious  that  the  anatomical  and  physiological  conditions 
do  not  necessarily  correspond :  as  a  nerve  which  is  structurally 
continuous  may  lose  its  conductivity  and  be  functionally 
destroyed  from  the  presence  of  various  local  causes  such 
as  callus  in  the  neighbourhood  of  a  fracture  or  cicatricial 
tissue. 

Symptomatology.  The  symptoms  of  peripheral  paralysis, 
whatever  the  cause,  may  be  motor,  sensory,  trophic,  and 
vaso-motor. 

Motor  symptoms :  - 

1.  Atrophic  paralysis  of  the  flaccid  type. 

2.  Abolition  of  the  tendon  reflexes. 

3.  Electrical  alterations  characteristic  of  the  reaction  of 
degeneration  (p.  51). 

4.  If  deformities  and  contractures  exist,  they  are  due  to 
the  over-action  of  non-paralysed  muscles.  These  deformities 
are  of  late  development. 

Sensory  symptoms.  The  sensory  symptoms  are  both 
subjective  and  objective.  The  former  consist  of  numbness, 
tingling,  '  pins-and-needles,'  or  of  sensations  of  burning  pain 
in  the  distribution  of  the  affected  nerve  or  plexus.  Sub- 
jective sensory  symptoms  are  usually  accompanied  by  pain  or 
tenderness  on  pressure  along  the  nerve  trunk.  On  the  other 
hand,  in  some  forms  of  compression  neuritis,  no  pain  or 
tenderness  may  be  present. 

The  objective  signs  depend  largely  upon  the  nature  of 
the  nerve  lesion.  In  acute  inflammatory  lesions,  sensory 
symptoms  are  well  marked ;  on  the  other  hand,  if  the  nerve 
is  injured  by  slow  compression,  the  motor  symptoms  are  early 
and  pronounced,  and  may  not  be  accompanied  by  sensory 
symptoms,  except  in  the  later  stages.  The  area  of  objective 
sensory  loss  resulting  from  an  isolated  nerve  lesion  is  not 
coterminous  with  the  anatomical  skin  distribution  of  the 
nerve,  because  of  the  overlap  existing  between  adjacent  nerve 
areas.  The  area  of  sensory  loss  is  therefore  contained  within 
the  anatomical  distribution  of  the  nerve,  and  consists  of  a 
larger  area  in  which  there  is  epicritic  loss,  and  a  less  extensive 
zone  of  protopathic  loss  (p.  14).  The  sensibility  to  contact 
and  deep  pressure  and  the  sense  of  passive  position  and  of 
movement  are  retained  if  the  cutaneous  branches  only  are 
involved.     In  the  area  between  the  margins  of   protopathic 


THE  PERIPHERAL  NERVOUS  SYSTEM  99 

and  epicritic  loss,  sensibility  is  modified  in  that  painful 
stimuli  cause  greater  discomfort  than  normal,  and  the  pain 
which  results  is  widely  diffused  and  cannot  be  localised. 

Trophic  symptoms.  These  consist  of  atrophy  of  the  skin, 
which  becomes  thin  and  glossy ;  the  development  of  ulcers 
and  sores ;  atrophy  of  bones  and  joints,  and  falling  out 
of  the  hair  and  nails.  According  to  Head  these  changes 
are  associated  with  loss  of  the  protopathic  sensibility.  They 
are  therefore  only  present  when  the  nerve  is  severely  affected. 
As  in  slight  injuries  and  minor  degrees  of  neuritis,  the  sensory 
loss  may  consist  merely  of  epicritic  anaesthesia. 

Recovery  of  sensation  after  injury  to  nerves,  or  after 
reunion  of  a  divided  nerve,  takes  place  first  in  the  protopathic 
and  subsequently  in  the  epicritic  sensibilities.  With  the 
restitution  of  the  former,  there  is  a  rapid  recovery  and 
healing  of  ulcers,  sores,  and  other  trophic  phenomena 
(Head). 

Vaso-motor  and  secretory  symptoms.  There  may  be  redness 
of  the  skin  with  local  rise  of  temperature  and  hyperidriosis. 
In  the  later  stages  blueness,  diminution  of  local  temperature, 
and  dryness  of  the  skin  are  present.  (Edema  is  not  a 
common  feature. 

Diagnosis.  A  differential  diagnosis  has  to  be  made 
between  peripheral  nerve  lesions  and  Arthritic  muscular 
paralysis  arising  from  lesions  of  joints.  In  the  latter,  only 
minor  degenerative  electrical  changes  are  detected  in  the 
muscles;  there  is  no  sensory  loss  or  impairment,  and  the 
distribution  of  the  paralysis  is  in  direct  relation  to  the 
joint  injury  and  not  to  that  of  any  nerve. 

Another  condition  requiring  to  be  distinguished  from 
peripheral  nerve  lesion  is  Ischcemic  paralysis.  This  results 
from  the  too  tight  application  of  bandages  or  splints,  causing 
swelling  of  the  free  extremity  of  the  limb,  associated  with 
great  pain  and  tenderness.  At  a  later  stage  there  are  rigidity 
and  hardness  of  the  muscles,  loss  of  voluntary  power,  and 
limitation  of  passive  movement.  The  electrical  reactions  are 
normal  and  there  is  no  objective  sensory  loss. 

In  cases  where  a  nerve  is  paralysed  by  slow  compression, 
such  as  may  result  from  the  presence  of  a  cervical  rib  or  local 
overgrowth  of  bone,  the  condition  must  be  distinguished  from 
Syringomyelia.     In  pressure  paralyses  the  motor  and  sensory 


100  NERVOUS  DISEASES 

impairment  is  limited  to  the  distribution  of  the  affected 
roots  or  nerves  and  is  peripheral  in  type.  In  syringomyelia, 
the  paralysis  is  often  bilateral,  the  sensory  loss  dissociated 
and  characteristic  of  a  central  cord  lesion ;  and,  further, 
the  symptoms  progress  showing  involvement  of  other 
regions  of  the  cord  and  interference  with  the  spinal  tracts. 
In  all  doubtful  cases  an  X-ray  examination  should  be 
made. 

A  word  may  be  said  as  to  the  differential  diagnosis  from 
Hysterical  paralysis  with  contracture.  In  this  affection  there 
is  no  change  in  the  electrical  excitability  of  the  muscles,  and 
the  sensory  loss  shows  hysterical  features  described  on  a  later 
page  (p.  535).  Hysterical  contractures,  moreover,  differ 
essentially  in  appearance  from  those  found  in  peripheral 
lesions. 

Prognosis.  The  prognosis  as  regards  recovery  from 
peripheral  nerve  paralyses  depends  entirely  upon  the 
nature  of  the  lesion  and  the  degree  of  injury  to  the  nerve 
fibres. 

In  slight  cases — such  as  result  from  pressure,  blows,  or 
minor  degrees  of  traction,  in  which  the  electrical  excitability 
is  only  slightly  or  not  at  all  impaired — recovery  may 
be  expected  to  take  place  completely  and  within  a  few 
weeks. 

In  severer  cases — associated  with  the  reaction  of  degenera- 
tion and  wasting  of  muscles — progress  may  be  slow,  and 
recovery  only  occur  after  many  months. 

If  the  nerve  is  divided,  completely  or  incompletely,  or 
severely  lacerated;  excision  of  the  divided  ends  and  suture  of 
the  nerve  ought  to  be  undertaken.  An  important  question, 
and  one  often  hard  to  answer,  is  for  how  long  a  period  after 
the  receipt  of  an  injury  is  recovery  of  the  nerve  possible. 
According  to  Sherren,'  there  is  no  reason  why  good  results 
may  not  be  obtained  after  an  interval  of  three  or  even 
more  years.  In  cases  of  this  nature  the  condition  of  the 
paralysed  muscles  should  be  carefully  investigated,  as  being 
more  likely  to  influence  the  prognosis  than  any  other  factor. 
If  galvanic  and  mechanical  excitability  of  the  muscles 
are  abolished,  recovery  of  muscular  power  is  highly 
improbable. 

1  Sherren,  Injuries  of  Nerves.  1909. 


THE  PERIPHERAL  NERVOUS  SYSTEM  101 

Sherren  recommends  re-suture  of  the  nerves,  even  in 
cases  in  which  a  return  of  motor  power  is  unlikely, 
with  a  view  to  the  restoration  of  protopathic  sensibility, 
and  the  consequent  resolution  of  trophic  sores  and 
ulcers. 

In  cases,  on  the  other  hand,  in  which  the  galvanic  irrita- 
bility is  retained,  although  polar  changes  may  be  present, 
re-suture  of  the  nerve  should  always  be  undertaken. 

If  the  suture  be  successful,  recovery  takes  place  in  the 
following  order : — 

(a)  Protopathic  sensibility  begins  to  return  in  from  six 
to  sixteen  weeks,  and  is  complete  in  from  four  to  twelve 
months. 

(6)  Epicritic  sensibility  is  re-established  in  from  twelve  to 
eighteen  months. 

(c)  Recovery  of  the  motor  functions  varies,  being  more 
rapid  the  nearer  the  lesion  is  to  the  periphery  of  the  limb ; 
the  time  required  is  from  one  to  two  years. 

Treatment.  The  treatment  of  nerve  paralyses  is  conducted 
upon  general  lines.  In  the  first  place,  it  is  necessary  to  main- 
tain the  nutrition  of  the  muscle  fibres.  This  is  partly  done 
by  means  of  the  faradic  or  galvanic  currents,  according  to 
the  form  of  electrical  stimulation  to  which .  the  muscles 
respond  ;  and  partly  by  massage  and  passive  movements  at 
the  joints.  So  far  as  the  muscular  weakness  permits,  these 
may  be  aided  by  movements  against  resistance. 

Secondly,  deformities  and  contractures  of  the  paralysed 
parts  should  be  prevented  by  passive  movements  at  the 
joints,  as  already  mentioned,  and  by  the  aid  of  carefully 
adjusted  splints,  and  apparatus  designed  to  aid  the  action 
of  paralysed  muscles  and  to  prevent  the  over-action  of  the 
non -paralysed. 

Thirdly,  care  should  be  taken  to  prevent  accidental  bruises, 
pressure  over  bony  prominences,  and  the  contact  of  substances 
which  may  irritate  the  skin. 

Fourthly,  suture  of  the  injured  nerve  should  be  undertaken 
in  suitable  cases,  and  treatment  on  the  above-mentioned  lines 
carried  out  until  the  return  of  the  sensory  and  motor  functions 
renders  it  unnecessary. 

In  cases  of  slow  compression,  as  in  cervical  rib,  the 
treatment  consists  of  removal  of  the  cause. 


102 


NERVOUS  DISEASES 


Lesions  of  Special  Nerves 
The  Phrenic  Nerve 

The  phrenic  nerve  arises  from  the  third,  fourth,  and  fifth 
cervical  nerves  and  supplies  the  diaphragm. 

The  phrenic  nerve  may  be  paralysed  by  lesions  of  the  third 
and  fourth  cervical  segments  and  by  implication  of  the  third 
and  fourth  cervical  nerve  roots  from  trauma,  hemorrhage, 
syphilitic    meningitis,  tumours,    tuberculous    and   malignant 


Fig.  29. — Shows  the  winging  of  the  scapula  in  a  case  of  paralysis 
of  the  right  serratus  magnus  muscle.  Both  arras  are 
extended  horizontally  in  front  of  the  patient. 

disease  of  the  vertebrae.  It  may  be  involved  in  its  course 
through  injury,  or  from  tumours  situated  in  the  neck  or 
thorax,  but  such  causes  are  rare.  It  may  also  be  implicated 
in  neuritis,  especially  in  the  post-diphtheritic,  alcoholic,  and 
toxic  varieties,  but  rarely  in  lead  neuritis. 

The  resulting  paralysis  is  shown  by  an  absence  of  descent 
of  the  diaphragm  on  inspiration,  more  particularly  on  deep 
inspiratory  efforts.    If  the  paralysis  is  complete,  there  may  be 


THE  PERIPHERAL  NERVOUS  SYSTEM 


103 


retraction  of  the  epigastric  region  and  the  lower  border  of  the 
liver.  In  expiration,  a  bulging  of  the  hypochondria,  protru- 
sion of  the  stomach,  and  downward  displacement  of  the  liver 
are  observed.  Even  slight  exertion  may  be  accompanied 
by  considerable  breathlessness  and  distress.  Expectoration, 
coughing,  and  defalcation  may  also  be  hindered.  Unilateral 
paralysis  gives  rise  to  little  discomfort. 

Prognosis.  This  is  largely  dependent  upon  the  cause  of 
the  paralysis.  Its  occurrence  as  a  complication  is  serious  in 
multiple  neuritis,  but  not  necessarily  fatal ;  and  in  the  non- 
fatal cases,  complete  recovery  usually  occurs. 


The  Long  Thoracic  Nerve 

This  nerve  arises  from  the  fourth,  fifth,  and  sixth  cervical 
nerves,  and  .supplies  the  serratus  maguus  muscle. 

It  is  paralysed 
alone  as  a  result  of 
traumatism,  common 
causes  of  which  are  in- 
juries to  the  shoulder 
and  wounds  in  the 
axilla  and  supra- 
scapular region,  from 
carrying  weights  upon 
the  shoulder  and 
from  over -exertion, 
especially  of  elevation 
of  the  arm.  It  is  also 
paralysed  in  acute 
infective  disorders. 
Its  palsy  is  more 
common  on  the  right 
side  than  the  left,  and 
more  frequent  in  men 
than  in  women. 

A  characteristic 
picture  is  presented 
by   paralysis    of    the 

serratus  magnus.  When  the  arm  is  at  rest  the  scapula  is 
higher  than  normal,  and  its  inner  border  is  approximated  to 
the   vertebrae,  especially   at   its   lower   end,   so   that   it   lies 


Fig.   30. — Paralysis   of  the  serratus  magnus 
muscle,  viewed  from  the  side. 


104  NERVOUS  DISEASES 

obliquely  upwards  and  outwards.  On  carrying  the  arm 
forwards,  the  inner  border  of  the  scapula  wings  outwards 
from  the  chest.  If  the  arm  is  abducted  into  the  horizontal 
position,  the  shoulder  blade  approaches  the  vertebral  column, 
its  inner  border  is  raised,  and  the  rhomboids  and  trapezius 
muscles  are  displaced  backwards.  The  arm  cannot  be  raised 
above  the  horizontal,  as  from  the  lack  of  scapular  rotation 
the  necessary  fixation  of  the  shoulder  does  not  take  place.  If 
the  arm  is  carried  forwards,  pressure  applied  on  it  in  a  back- 
ward direction  causes  displacement  of  the  shoulder  blade. 
(Figs.  29  and  30.) 

The  paralysis  is  accompanied  by  pain  in  the  neck  and 
shoulder,  but  no  objective  sensory  loss  is  present. 

The  prognosis  is  on  the  whole  good,  but  recovery  may  be 
delayed  for  many  months. 


Chapter  II 

PARALYSES  OF  THE  UPPER  LIMB 

Paralysis  of  the  nerves  of  the  upper  limb  may  be 
described  according  as  the  lesion  involves  :— 

1.  The  nerve  roots. 

2.  The  brachial  plexus. 

3.  The  individual  nerves. 

1.  Lesions  of  the  Roots  of  the  Brachial  Plexus 

The  motor  symptoms  of  lesion  of  the  nerve  roots  of  the 
upper  limb  correspond  to  those  following  disease  or  destruction 
of  the  related  segments  of  the  spinal  cord,  and  are  recorded 
on  a  subsequent  page  (p.  317). 

The  sensorij  loss  covers  a  definite  area  of  skin  over  the  hand, 
forearm,  and  upper  arm,  and  has  been  accurately  delimitated 
after  division  of  the  posterior  roots  of  the  brachial  plexus. 

The  accompanying  chart  (fig.  31)  shows  the  distribution 
of  the  epicritic  and  protopathic  loss  following  division  of  the 
posterior  roots  from  the  sixth  cervical  to  the  second  dorsal 
inclusive.  It  will  be  observed  that  the  area  of  protopathic  is 
larger  than  that  of  epicritic  loss. 


THE  PERIPHERAL  NERVOUS  SYSTEM 


105 


2.  Lesions  op  the  Brachial  Plexus 
The   brachial   plexus  is  formed   by   the  anterior  primary 

divisions  of  the  five  nerves  from  the  fifth  cervical  to  the  first 

dorsal    inclusive.       The   fifth 

and    sixth    nerves  constitute 

the  upper  trunk,  the  seventh 

the    middle    trunk,    and    the 

eighth  cervical  and  first  dorsal 

the  lower  trunk  of  the  plexus. 

(Fig.  32.) 

Etiology.     The  chief  cause 

of    lesion     of     the    brachial 

plexus  is    undue   violence  or 

traction  applied    to  the  arm, 

neck,  or    shoulder.      Of   this 

nature     are     the    obstetrical 

paralyses,  arising  from  trac- 
tion upon  the  arm  during  the 

process  of  delivery,  paralyses 

arising    from    traction    upon 

the  arms  in  connexion  with 

injuries    about    the    shoulder 

joint,  and  paralyses  following 

operations    upon    the    breast 

and  axilla,  when   the  arm  is 

forcibly  abducted  and  main- 
tained     in      an      unnatural 

position.     Gunshot  wounds  or 

other    accidental    injuries    of 

the    plexus    are    rare.      The 

plexus  may  also  be  implicated 

in  dislocations  of  the  humerus, 

and  by  tumours  or  aneurismal 

dilatation    in    the     posterior 

triangle  of  the  neck.     A  toxic 

or   infective   neuritis   of    the 

plexus,  though  rare,  has  also 

been  described. 

Traction     injuries    of    the 

plexus  are  due  to  tearing  of  the  nerve    sheaths,    sometimes 

accompanied  by  hemorrhage  within  the  sheath,  and  in  severe 


Fig.  31. — Area  of  sensory  loss  follow- 
ing section  of  the  posterior  nerve 
roots  from  the  sixth  cervical  to 
the  second  dorsal  inclusive.  The 
black  area  corresponds  to  the 
zone  of  protopathie,  epicritic.  and 
deep  sensory  loss ;  the  shaded 
represents  the  overlap  of  proto- 
pathie loss. 


106 


NERVOUS  DISEASES 


instances  to  an  actual  rupture  of  the  nerve  fibres.  In  less 
severe  cases,  however,  the  lesion  would  appear  to  consist  merely 
of  over-stretching  of  the  nerves  without  any  serious  solution 
of  their  continuity. 


Fifth 


Sixth  a  roical. 


Seventh  cervical. 

To    seal  eni    and 

lonaus  colli. 
Eighth  cervical. 


Long  thoracic. 
First  thoracic. 


First  intercostal. 
Second  thoracic. 


Second  intercostal. 
Third  thoracic. 


Third  intercostal. 


-  From  fourth  cervical 


-Dorsal  scapular 


Supra-sea p  a  I nr. 
^Anterior  thoracic  nerves. 


Outer  cord  of  Plexus. 
Axilliary  (circumflex). 

Mnsculo-cntaneous. 
Radial  (m  \isculo-spiral  I. 
Inner  cord  of  Plexus. 

Thoraco-dorsal. 
Median. 


I'limr. 

Medial  aiM-brachial 
cutaneous. 

Medial  brachial  cu- 
taneous (nerve  of 
Wri&bero). 


Intercosto-brachial.  Lateral  cutaneous. 

Pig.  32  (from  Morris's  'Anatomy  ')• — Diagram  of  a  common  form  of  brachial 
plexus.     The  posterior  cord  of  the  plexus  is  darkly  shaded. 

In  obstetrical  paralysis,  and  in  paralysis  resulting  from 
malposition  of  the  arm  during  operations,  the  upper  trunk 
of  the  plexus  is  more  commonly  the  seat  of  lesion.  On  the 
other  hand,  the  lower  trunks  receive  the  chief  brunt  of  the 
traction  when  the  arm  is  outstretched,  as  in  trying  to  save 
the  body  when  falling  from  a  height. 

Symptoms.  The  symptoms  of  lesion  of  the  brachial  plexus 
may  be  described  according  as  they  are  due  to  interference 
with  : — 

(1)  The  whole  plexus. 

(2)  The  upper  trunk. 

(3)  The  middle  trunk. 

(4)  The  lower  trunk. 

(•';)  The  cords  of  the  plexus. 


THE  PERIPHERAL  NERVOUS  SYSTEM 


107 


(i)  The  whole  brachial  plexus 

In  lesions  of  the  whole  brachial  plexus  the  entire  muscu- 
lature of  the  arm  is  paralysed,  with  the  exception  of  the 
serratus  magnus,  rhomboids,  and  levator  anguli  scapula.  In 
most  cases  the   pectoral  muscles  are   also   paralysed.     The 


Fig.  33. — The  distribution    of  the  sensory   loss    after  lesion    of  the  brachial 

plexus   (Head). 


paralysis  rarely  remains  total,  and  the  residual  paralysis 
presents  the  features  of  either  the  upper  or  lower  arm 
type. 

According  to  Sherren  the  sensory  symptoms  consist  of 
epicritic  and  protopathic  loss  over  the  whole  of  the  forearm 
and  hand,  and  the  outer  surface  of  the  upper  arm  in  its  lower 
two-thirds.  The  tip  of  the  shoulder  and  the  inner  surface  of 
the  upper  arm  are  sensitive,  owing  to  the  intact  condition  of 


108 


NERVOUS  DISEASES 


the  descending  cutaneous  branches  of  the  cervical  plexus 
and  of  the  mtercosto-humeral  nerve  (fig.  33).  The  sympa- 
thetic nerve  is  usually  paralysed. 

Total  palsy  is   a  serious   affection,  and  complete  recovery 
rarely  occurs. 


(2)  The  upper  trunk 

The  upper  trunk  contains  the  fibres  of  the  fifth  and 
sixth  cervical  nerves,  with  the  exception  of  the  nerves  to 
the  serratus  magnus,  rhomboids,  and  levator  angulis  capulae 

muscles,  which  are 
given  off  before  the 
trunk  is  formed. 
A  lesion  of  the 
upper  trunk  gives 
rise  to  an  extensive 
paralysis  of  the 
shoulder  and  arm 
movements,  especi- 
ally those  depend- 
ing on  the  action 
of  the  teres  minor, 
pectoralis  major, 
deltoid,  supra-  and 
infra-spinatus, sub- 
scapulars, biceps, 
brachialis  anticus, 
and  supinator 
longus,  and  some- 
times the  extensors 
of  the  wrist. 

Lesion  of  this 
trunk  gives  rise  to 
the  upper  arm 
type  of  paralysis,  or  Erb's  palsy,  which  is  found  as  the 
type  of  the  obstetrical  paralyses,  and  of  paralyses  consequent 
upon  malposition  of  the  arm  in  operations  upon  the  breast. 
It  may  also  form  the  residual  paralysis  after  a  lesion  of  the 
whole  plexus. 

The  arm  hangs  flaccid  by  the  side,   with  the  forearm   in 


Fig.  34  —  Erb's  palsy  from  lesions  of  the  upper 
trunk  of  the  brachial  plexus.  The  figure 
shows  an  attempt  to  elevate  the  paralysed 
arm. 


THE  PERIPHERVL  NERVOUS  SYSTEM  109 

pronation.  The  arm  cannot  be  elevated  or  abducted  at  the 
shoulder  joint.  Flexion  and  supination  of  the  forearm  are 
in  abeyance.  In  old-standing  cases  much  wasting  of  the 
paralysed  muscles  is  observed.  In  most  cases  there  is  no 
sensory  impairment,  although,  according  to  Sherren,  some 
diminution  or  loss  of  epicritic  sensibility  may  be  detected  over 
the  outer  surface  of  the  arm  and  forearm. 

The  prognosis  depends  upon  the  cause  of  the  paralysis. 
In  the  obstetrical  cases  recovery  is  usually  rapid  and  satis- 
factory, although  complete  resolution  is  not  always  obtained. 
In  the  post -operative  cases  recovery  usually  occurs,  but  the 
paralysis  may  persist  for  many  months,  or  for  more  than 
a  year. 

(3)  The  middle  trunk 

This  trunk,  which  corresponds  mainly  to  the  seventh 
cervical  segment,  contains  fibres  for  the  triceps,  the  pronators, 
and  the  extensors  and  flexors  of  the  wrist  and  fingers.  It 
may  be  paralysed  in  lesions  of  the  whole  plexus.  Paralysis 
of  some  of  the  muscles  supplied  by  it— viz.,  the  extensors  of 
the  wrist  and  fingers — may  form  a  part  of  the  Brb,  or  upper 
arm  type  of  palsy,  while  palsy  of  the  flexors  of  the  wrist  and 
fingers  may  be  an  element  of  the  lower  arm  type,  next  to  be 
described.  There  would  not  appear  to  be  a  clinical  type  of 
paralysis,  whose  symptoms  point  to  an  isolated  lesion  of  this 
trunk. 

(4)  The  lower  trunk 

The  lower  trunk  of  the  plexus  is  formed  by  the  eighth 
cervical  and  first  dorsal  nerves,  and  conveys  fibres  for  the 
intrinsic  muscles  of  the  hand  and  the  thenar  and  hypothenar 
eminences.  It  may  form  part  of  a  total  plexus  paralysis, 
of  which  it  may  be  the  residual  palsy.  On  the  other  hand, 
it  may  be  paralysed  alone,  in  which  case  it  forms  the  lower 
arm  type  of  brachial  plexus  palsy,  or  KlumpJce  paralysis.  Its 
paralysis  forms  the  chief  feature  of  the  symptomatology  of 
a  cervical  rib  (p.  110). 

There  is  paralysis  with  wasting  of  all  the  intrinsic  muscles 
of  the  hand,  which  presents  a  claw-shaped  appearance.  The 
flexors  of  the  fingers  are  also  occasionally  paralysed. 


110  NERVOUS  DISEASES 

Sensation,  both  the  epicritic  and  protopathic  varieties,  is 
impaired  along  the  ulnar  border  of  the  hand  and  forearm. 

In  some  cases  an  associated  palsy  of  the  sympathetic 
fibres  is  demonstrable.  "When  this  occurs,  the  nerve  roots 
have  probably  been  torn  or  otherwise  injured  near  the 
spinal  cord. 

Cervical  ribs.     The  symptoms  occasioned  by  the  presence 


Fig.  35. — Showing  the  atrophy  of  the  hand  and  forearm  in  a  case  of 
cervical  rib  on  the  right  side. 

of  a  supernumerary  or  cervical  rib  may  be  explained  as  the 
result  of  pressure  upon  the  lower  trunk  of  the  plexus.  In 
this  condition  the  motor  symptoms  consist  mainly  of  paralysis 
and  atrophy  of  the  intrinsic  muscles  of  the  hand,  with  an 
anaesthetic  area  along  the  ulnar  border  of  the  forearm  and 
hand.  Subjective  sensations  of  pain,  often  of  a  severe 
character,  with  numbness  and  tingling,  are  also  described. 
The  absence  of  symptoms  pointing  to  lesion  of  the  sympathetic 
fibres  (narrowing  of  the  palpebral  fissure  and  constriction  of 


THE  PERIPHERAL  NERVOUS  SYSTEM  111 

the  pupil)  would  appear  to  place  the  lesion  in  the  lower  trunk 
rather  than  in  the  roots  of  the  plexus.     (Fig.  35.) 

The  condition  is  more  commonly  met  with  clinically  in 
women  than  in  men,  and  more  often  affects  the  right  side. 
The  symptoms  do  not  usually  appear  until  early  adult  life. 

The  diagnosis  is  rendered  especially  easy  by  the  use  of  the 
Eontgen  rays,  and  the  treatment  consists  in  removing  the 
exostosis  or  additional  rib. 

(5)  The  cords  of  the  brachial  plexus 

Each  trunk  of  the  brachial  plexus  divides  into  an  anterior 
and  a  posterior  branch.  All  the  posterior  branches  unite 
to  form  the  posterior  cord,  the  anterior  branches  of  the 
upper  and  middle  trunks  unite  to  form  the  outer  cord,  while 
the  anterior  branch  of  the  lower  trunk  forms  the  inner  cord 
(see  fig.  32).  In  this  way  an  important  rearrangement  of 
the  nerve  fibres  in  the  trunks  of  the  plexus  is  brought  about. 

The  outer  cord  of  the  plexus  contains  the  fibres  of  the 
upper  trunk,  with  the  exception  of  those  for  the  supra-  and 
infra- spinatus  muscles.  It  receives  from  the  middle  trunk 
the  nerve  fibres  for  the  pronators  and  long  flexors  of  the 
wrist  and  fingers.  It  eventually  gives  origin  to  the  external 
anterior  thoracic  nerve,  the  musculo-cutaneous  nerve,  the 
nerve  to  the  coraco-brachialis,  and  the  outer  head  of  the 
median.  The  symptoms  of  lesion  of  the  outer  cord,  therefore, 
consist  of  paralysis  of  the  pectoralis  major,  the  biceps, 
brachialis  anticus  (partly),  the  coraco-brachialis,  the  pronator 
teres,  flexor  carpi  radialis,  and  flexor  sublimis  digitorum. 
Sensation  is  impaired  over  an  area  on  the  outer  surface 
of  the  forearm,  whose  anterior  boundary  is  well  defined, 
but  whose  posterior  boundary  fades  into  the  area  supplied  by 
the  descending  branch  of  the  musculo-spiral  nerve  (Sherren). 

The  posterior  cord  of  the  plexus,  in  addition  to  containing 
some  of  the  nerve  fibres  of  the  middle  trunk,  receives 
fibres  from  the  upper  trunk  for  the  deltoid,  teres  minor,  and 
sub-scapularis  muscles,  and  also  some  from  the  lower  trunk. 
It  gives  origin  to  the  subscapular,  circumflex,  and  musculo- 
spiral  nerves.  The  symptoms  of  lesion  of  the  posterior 
cord  are  as  follows  :  paralysis  of  the  subscapularis,  of  the 
deltoid  and  teres  minor,  of   the   triceps,  brachialis   anticus 


112 


NERVOUS  DISEASES 


(partly),  supinators,  long  and  short  extensors  of  the  wrist  and 
lingers,  and  the  extensors  of  the  thumb  and  index  ringer. 

The  skin  areas  affected  correspond  to  the  cutaneous 
distribution  of  the  circumflex  and  musculo-spiral  nerves 
elsewhere  described. 


Fig.  36. — The  area  of  sensory  loss  following  lesion  of  the  inner  cord  of  the 
brachial  plexus  (Head).  The  front  of  the  hand  and  forearm  is  shown 
on  the  left,  the  back  on  the  right  side  of  the  figure. 


The  inner  cord  of  the  plexus  contains  the  remaining  nerve 
fibres  of  the  lower  trunk.  It  gives  origin  to  the  internal 
anterior  thoracic,  the  internal  cutaneous,  the  inner  head  of 
the  median  and  the  ulnar  nerves.  A  lesion  is  characterised 
by  paralysis  of  the  pectoralis  major,  flexor  profundus  digitorum, 
flexor  longus  pollicis,  flexor  carpi  ulnaris,  the  lumbricales  and 


THE  PERIPHERAL  NERVOUS  SYSTEM  113 

int.erossei,  and  the  special  muscles  of  the  thumb  and  little 
linger.  The  sensory  loss  corresponds  to  the  area  of  ulnar 
anaesthesia  upon  the  inner  side  of  the  forearm  and  hand. 
(Fig.  36.) 

The  Circumflex  Nerve 

This  nerve  arises  from  the  posterior  cord  of  the  brachial 
plexus,  and  supplies  the  deltoid  and  teres  minor  muscles  ;  it 
also  sends  branches  to  the  shoulder  joint. 

The  commonest  cause  of  paralysis  is  a  fall  on  or  injury  to 
the  shoulder,  especially  with  dislocation  of  the  shoulder  joint. 
Amongst  general  causes,  lead  poisoning  and  diabetes  may 
induce  an  isolated  palsy.  This  may  also  occur  in  typhoid 
fever. 

As  the  muscles  are  primarily  concerned  in  elevation  and 
outward  rotation  of  the  upper  arm,  these  movements  are 
paralysed  in  lesions  of  the  circumflex.  There  is  an  area 
of  epicritic  and  protopathic  loss  over  the  insertion  of  the 
deltoid,  and  the  upper  half  of  the  outer  side  of  the  arm. 
Arthritic  changes  in  the  shoulder  joint  are  sometimes  found. 

It  is  commonly  affected  along  with  the  supra-scapular 
nerve,  which  arises  from  the  upper  trunk  of  the  plexus,  and  is 
distributed  to  the  supra-  and  infra-spinatus  muscles.  Its  skin 
area  corresponds  to  the  outer  border  of  the  scapula,  contiguous 
with  that  of  the  circumflex. 


The  Musculo-Spiral  Nerve 

This  nerve  arises  from  the  posterior  cord  of  the  brachial 
plexus  and  supplies  the  following  muscles  :  triceps,  brachialis 
anticus,  supinator  longus,  extensor  carpi  radialis  and  ulnaris, 
extensor  communis  digitorum,  and  the  extensors  of  the  thumb 
and  little  finger.  Through  its  radial  and  external  cutaneous 
branches  it  supplies  the  skin  on  the  radial  side  of  the  back  of 
the  wrist,  thumb,  and  hand. 

Etiology.  Paralysis  of  the  musculo-spiral  nerve  is  an 
important  feature  in  lesion  of  the  posterior  cord  of  the  brachial 
plexus  (p.  Ill),  in  which  case  the  circumflex  and  subscapular 
nerves  are  also  involved. 

Isolated  paralysis  of  the  nerve  is  common.  It  may  be 
implicated  in  the  upper  part  of  the  arm  by  the  pressure  of 


114  NERVOUS  DISEASES 

a  crutch,  by  traction  on  the  arm  and  in  the  '  sleep  palsies  ' 
following  alcoholic  intoxication.  In  these  cases  the  lesion  is 
above  the  origin  of  the  nerve  to  the  triceps  muscle.  Below  the 
origin  of  this  nerve  the  chief  causes  of  palsy  are  fractures  in 
the  lower  third  of  the  arm,  gunshot  or  other  wounds,  disloca- 
tion of  the  head  of  the  radius  and  operations  upon  the  arm. 

The  nerve  may  also  be  paralysed  from   infective  causes 
during  the  course  of  enteric  fever,  the  puerperium  and  articular 


Fig.  37. — Showing  drop  wrist  in  a  case  of  musculo-spiral  paralysis. 
The  prominence  on  the  dorsum  of  the  wrists  is  well  seen  in  the 
photograph. 

rheumatism.  Paralysis  of  the  nerve  also  forms  an  element  in 
the  multiple  peripheral  paralyses  of  lead,  arsenic,  and  alcoholic 
neuritis.  In  the  case  of  lead  palsy  the  supinator  longus 
muscle  is  rarely  affected — a  point  of  some  importance  in 
differential  diagnosis. 

Symptoms.  The  motor  symptoms  are  seen  in  wrist  and 
fingerdrop,  and  in  the  event  of  the  lesion  being  above  the 
origin  of  the  nerve  to  the  triceps,  in  inability  to  extend  the 
forearm  at  the  elbow.  The  appearance  of  the  hand  and 
forearm  is  characteristic.  The  patient  is  unable  to  extend  the 
wrist  or  the  fingers.  This  is  especially  observed  in  the  two 
ulnar  fingers,  and  least  in  the  index  finger.  Attempts  at 
wrist  extension  produce  some  degree  of  flexion,  owing  to  the 
unopposed  action  of  the  synergic  flexor  muscles.  There  is 
often  a  prominence  at  the  back  of  the  wrist  from  a  partial 
dislocation  of  the  carpus,  due  to  the  weakness  of  the  ex- 
tensors  and   the   stretching   of  the   dorsal   ligaments.     The 


THE  PERIPHERAL  NERVOUS  SYSTEM 


115 


Fig.  38. — Bilateral  drop  wrist  due  to  pressure 
on  both,  rnusculo-spiral  nerves  from 
the  use  of  crutches. 


supinator  longus  is  always  paralysed,  and  the  triceps  also, 
if  the  lesion  is  high  up  in  the  upper  arm.     (Figs.  37  and  38.) 

The  sensory  symptoms 
depend  upon  the  posi- 
tion of  the  lesion.  For 
the  purpose  of  studying 
the  sensory  loss  the 
nerve  may  be  divided 
into  three  parts  : — 

(a)  Lesion  above  the 
origin  of  the  external 
cutaneous  nerve.  When 
the  lesion  is  in  the  upper 
arm,  epicritic,  protopa- 
thic,  and  deep  sensibili- 
ties are  affected  over  a 
skin  area  on  the  dorsum 
of  the  hand  correspond- 
ing to  the  two  radial 
fingers,  and  upon  the 
back  and  outer  side  of 
the  thumb.  (Fig.  39.)  The  area  of  epicritic  is  more  extensive 
than  that  of  protopathic  loss. 

(b)  In  lesion  below  the  origin  of  the  external  cutaneous  branch 

(paralysis  of  the  radial 
nerve)  either  no  sensory 
impairment  is  detected ,  or 
only  epicritic  loss  upon  the 
outer  side  of  the  thumb. 

(c)  On  the  other  hand, 
lesion  of  the  external 
cutaneous  and  radial 
branches  is  followed  by  an 
epicritic  and  protopathic 
loss  corresponding  to  that 
described  after  lesion  of  the 
nerve  trunk  (Head),  but 
deep  sensibility  is  not 
affected. 

The  conclusions  to  be  drawn  from  these  observations  are, 
that  the  external  cutaneous  nerve  and  its  branches  are  the 


Fig.  39. — Loss  of  sensation  following  a 
lesion  of  the  radial  and  external 
cutaneous  branches  of  the  rnusculo- 
spiral  nerve.  The  total  area  of  loss 
is  contained  within  the  continuous 
thick  line.  The  black  is  the  area  of 
protopathic  and  epicritic  loss,  the 
zone  between  that  and  the  con- 
tinuous line  is  the  area  of  epicritic 
overlap.     (After  Head.) 


116  NERVOUS  DISEASES 

main  paths  for  both  epicritic  and  protopathic  sensibility  to 
the  skin  of  the  hand  supplied  by  the  rnusculo-spiral  nerve ; 
that  deep  sensibility  is  transmitted  b}r  the  posterior  interosseous 
nerve ;  and  that  the  radial  nerve  only  conveys  impressions 
of  epicritic  sensibility. 

The  prognosis  is  favourable  for  recovery  in  most  cases.  In 
the  simple  pressure  cases,  with  little  or  no  electrical  alterations, 
recovery  is  rapid.  If  the  reaction  of  degeneration  is  present, 
recovery  may  be  delayed  for  many  months.  In  gunshot 
wounds,  or  other  solutions  of  the  anatomical  continuity  of  the 
nerve  fibres,  resection  of  the  ends  of  the  nerve  and  suture  may 
be  necessary,  in  which  event  cure  may  not  ensue  until  after 
a  year,  even  in  favourable  cases ;  while  in  cases  complicated 
with  much  wasting  and  degenerative  changes,  complete 
recovery  may  never  take  place. 

The  treatment  is  based  on  the  general  indications  given 
on  p.  101.  A  glove,  designed  to  support  the  wrist  and  keep 
both  it  and  the  fingers  extended,  greatly  hastens  recovery,  and 
enables  the  patient  to  make  use  of  the  hand  in  grasping 
objects. 

The  Median  Nerve 

The  median  nerve  arises  by  two  heads  derived  from  the 
outer  and  inner  cords  of  the  brachial  plexus.  It  supplies  the 
following  muscles :  flexor  profundus  digitorum,  flexor  longus 
pollicis,  flexor  carpi  radialis,  flexor  sublimis  digitorum,  the  pro- 
nators, the  two  outer  lumbricals,  the  abductor  and  opponens 
pollicis,  and  the  short  head  of  the  flexor  brevis  pollicis. 

Etiology.  The  median  nerve  may  be  paralysed  from 
accidental  wounds  in  the  neighbourhood  of  the  wrist  joint, 
but  is  also  involved  in  other  parts  of  its  course  by  fractures 
in  the  upper  arm  or  forearm.  It  may  be  paralysed  in 
carpenters  and  professional  golfers  as  a  form  of  occupation 
or  craft  palsy. 

Symptoms.  The  symptoms  depend  upon  the  position  of 
the  lesion  whether  : — 

(a)  At  or  above  the  elbow. 

(b)  At  or  about  the  wrist. 

(a)  In  lesion  of  the  nerve  at  or  above  the  elbow  joint,  the 
motor  loss  is  chiefly  seen  in  weakness  and  immobility  of 
the  thumb  and  index  finger.     There  is  inability  to  flex  the 


THE  PERIPHERAL  NERVOUS  SYSTEM 


117 


terminal  phalanx  of  the  thumb,  or  to  flex  the  index  finger 
except  at  the  metacarpophalangeal  joint.  Pronation  is 
feeble. 

Epicritic  loss  corresponds  to  an  area  bounded  on  the 
palmar  surface  of  the  hand  by  a  line  drawn  through 
the  axis  of  the  ring  finger,  and  extending  to  the  limit  of 
musculo-spiral  loss  along  the  palmar  axis  of  the  thumb.  The 
line  then  passes  along  the  free  margin  of  the  first  interosseous 
space,  and  up  the  index  finger  until  it  reaches  the  first  inter- 
phalangeal  joint,  when  it  passes  across  the  middle  finger  and 
half  the  riug  finger  to  the  boundary  of  ulnar  anaesthesia  in 
the  axis  of  the  ring  finger.     (Fig.  40.) 

The  protopathic  loss 
varies  from  a  small  area 
over  the  tips  of  the  fingers 
to  one  almost  coextensive 
with  the  epicritic  anaes- 
thesia. Deep  sensibility 
is  retained, 

(b)  In  lesion  at  the 
wrist  joint,  the  motor 
symptoms  are  limited  to 
palsy  of  the  opponens  and 
abductor  actions  of  the 
thumb  and  of  the  two  outer  lumbrical  muscles.  The  sensory 
phenomena  correspond  to  those  of  lesion  at  or  above  the 
elbow  joint. 

Minor  injuries  of  the  median  nerve  are  apt  to  be  over- 
looked owing  to  the  absence  of  motor  deformity,  the  small 
protopathic  loss  and  the  retention  of  deep  sensibility 
(Sherren). 

The  prognosis  and  treatment  are  based  upon  the  general 
considerations  already  laid  down  on  pp.  100  and  101. 


Fig.  40. — Shows  the  area  of  sensory  loss 
following  lesion  of  the  median  nerve. 
The  shading  is  the  same  as  in  fig-.  39. 
(After  Head.) 


The  Ulnae  Nerve 

This  nerve  arises  from  the  inner  cord  of  the  brachial 
plexus,  and  supplies  the  following  muscles :  flexor  carpi 
ulnaris,  ulnar  half  of  the  flexor  profundus  digitorum,  interossei, 
two  inner  lumbricals,  adductor  and  half  the  flexor  brevis 
pollicis    and  the  muscles  of  the  hypothenar  eminence, 


118  NERVOUS  DISEASES 

Etiology.  Ulnar  paralysis  is  not  uncommon,  and  is  due 
to  injuries  and  wounds  about  the  elbow,  in  the  forearm,  and 
at  the  wrist.  It  may  occur  as  an  occupation  paralysis  in 
glass-workers  and  cigarette-makers,  and  it  is  found  in 
rheumatic  and  gouty  subjects  in  consequence  of  a  local 
neuritis. 

Symptoms.  These  may  be  described  according  as  the 
lesion  is  situated  :  — 

(a)   In  the  neighbourhood  of  the  elbow  joint. 

(b)  At  the  wrist  joint  below  the 
origin  of  the  dorsal  cutaneous  branch. 
(a)  Lesion  at  the  elbow  joint.  If 
the  lesion  is  above  the  origin  of  the 
muscular  branches  to  the  forearm, 
palsy  of  the  flexor  carpi  ulnaris  and 
the  ulnar  half  of  the  flexor  profundus 
digitorum  is  observed  in  addition  to 
paralysis  of  the  hand.  This  is  due 
to  palsy  of  the  interossei,  the  two 
inner  lumbricals,  and  the  adductor  of 
the  thumb.  The  appearance  of  the 
hand  is  characteristic.  The  little  and 
ring  fingers  are  hyper-extended  at 
the  metacarpophalangeal  joints ;  all 
the  fingers,  but  especially  the  little 
and  ring  ringers,  are  flexed  at  the 
inter-phalangeal  joints.  Abductor 
Fig.  41. — Showing  the  ap-  and  adductor  action  of  the  ringers  is 
afteTTnimy  To  the  ^os^>  adduction  of  the  thumb  is  also 
ulnar  nerve.  in  abeyance,  although  a  false  adduc- 

tion is  possible  by  aid  of  the  flexor 
longus  pollicis  and  the  extensors  of  the  thumb  (Sherren). 
The  interosseus  spaces  are  well  defined  owing  to  wasting 
of  the  interossei  muscles.  The  hand  may  also  be  tilted 
somewhat  to  the  radial  side.     (Fig.  41.) 

The  sensory  symptoms  consist  of  epicritic  loss  over  the 
little  and  ulnar  half  of  the  ring  finger  and  a  corresponding 
area  over  the  palm  and  back  of  the  hand,  as  far  as  the  wrist. 
The  epicritic  loss  is  in  excess  of  the  protopathic,  which  is 
variable.  Loss  of  deep  sensibility  corresponds  to  the  area  of 
protopathic  anaesthesia.     (Figs.  42  and  43.) 


THE  PERIPHERAL  NERVOUS  SYSTEM 


110 


(b)  Lesion  at  the  wrist  below  the  origin  of  the  dorsal 
cutaneous  branch.  The  motor  symptoms  are  seen  in  palsy 
of  all  the  intrinsic  muscles  of  the  hand  supplied  by  the  ulnar 
nerve.     The  area  of  epicritic  loss  corresponds  to  that  seen 


Fig.   42. — Loss  of  sensation  follow-  Fig.  43. — 'Showing  the  loss  of  sensa- 

ing  a  lesion  of  the  ulnar  nerve.  tion  following  lesion  of  the  ulnar 

The     total     area     is     contained  and     median      nerves.        (After 

within  the  continuous  black  line.  Head.) 
The  shading  is  the   same  as  in 
Bg.  39.     (After  Head.) 

after  lesion  of  the  whole  nerve.  The  area  of  protopathic 
loss  varies,  but  is  usually  less  extensive  than  that  seen 
after  lesion  of  the  nerve  higher  up,  and  deep  sensibility  is 
retained. 

The  prognosis  and  the  treatment  are  referred  to  in  detail 
on  pp.  100  and  101. 


Chapter    III 


PARALYSES    OF    THE   LOWER    LIMB 


Root  Lesions — Paralysis  op  the  Cauda  Equina 

The  cauda  equina  is  formed  by  the  nerve  roots  of  the 
lumbo- sacral  segments  of  the  cord.  As  in  the  adult  the 
spinal  cord  ends  at  the  level  of  the  first  lumbar  vertebra, 
the  nerve  roots  have  to  pass  for  a  considerable  distance 
within  the  neural  canal  before  they  reach  their  points  of  exit 
at  the  corresponding  intervertebral  foramina.  As  each  pair 
of  foramina  is  reached,  the  corresponding  nerve  roots  separate 
from  the  cauda  equina  and  pass  out  of  the  neural  canal. 
Thus  the  upper  lumbar  roots  are  situated  peripherally  and 
run  a  relatively  short   course  within  the  canal,  while  the 


120  NERVOUS  DISEASES 

sacral  roots  are  placed  more   mesially  and   have  a  longer 
course. 

The  conus  terminalis,  or  extreme  lower  end  of  the  spinal 
cord,  is  defined  anatomically  as  that  portion  of  the  cord 
which  lies  below  the  level  of  the  second  sacral  segment.  As 
the  conus  is  surrounded  by  the  nerve  roots  from  the  second 
lumbar  segment  downwards,  it  follows  that  lesions  of  the 
upper  part  of  the  cauda  equina  may  also  involve  the  conus 
terminalis.  It  does  not  contain  any  pyramidal  fibres  below 
the  level  of  the  fourth  sacral  segment,  but  contains  the  centres 
for  erection  of  the  penis,  and  the  anal  and  vesical  sphincters, 
although,  according  to  Miiller,1  the  reflex  centres  for  micturition 
and  defalcation  are  found  in  the  pelvic  sympathetic  ganglia. 

Etiology.  The  nerve  roots  forming  the  cauda  equina  may 
be  implicated  by  traumatic  lesions  of  the  spine,  by  an  exten- 
sion of  malignant  or  tuberculous  disease  from  the  bones  of 
the  lumbar  and  sacral  regions,  by  meningitis — especially  the 
gummatous  variety — and  by  new  growths,  mainly  sarcomata, 
neurofibromata,  endotheliomata,  and  angeiomata. 

Symptoms.  The  symptoms  of  lesion  of  the  cauda  equina 
vary  in  their  mode  of  onset  and  course  according  to  the 
character  and  situation  of  the  lesion. 

In  traumatic  lesions  the  onset  of  paralysis  may  be 
immediate  owing  to  direct  injury  to  the  roots,  or  may  follow 
shortly  after  from  secondary  effects,  such  as  hemorrhage  or 
suppuration.  In  these  cases  the  symptoms  are  referred  to 
the  motor,  sensory,  and  sphincter  functions. 

Where  the  lesion  is  due  to  chronic  meningitis  or  new 
growth,  the  symptoms  are  of  gradual  onset,  at  first  limited  to 
certain  nerve-root  areas  and  often  affecting  the  motor,  sensory, 
or  sphincter  functions  separately.  The  situation  of  the  lesion 
determines  the  extent  and  the  area  of  the  paralysis.  If 
situated  at  the  lower  end,  sphincter  paralysis  and  sensory 
changes  in  the  perinseal  region  are  alone  observed.  If  situated 
higher  up  and  peripherally,  motor,  sensory,  or  motor  and  sensory 
paralyses  in  the  distribution  of  the  affected  root  areas  will  be 
found  without  any  sphincter  weakness.  Should  the  lesion  be 
situated  high  up  and  central  in  position,  severe  paralysis, 
motor  and  sensory,  will  be  present  in  the  distribution  of  the 
sacral  and  lower  lumbar  roots,  with  complete  sphincter 
1   Miiller,  Dcutsch.  Zeitsch.  f.  Nervenlieilk.  1901 


THE  PERIPHERAL  NERVOUS  SYSTEM  121 

paralysis.  The  motor  symptoms  are  :  atropine  flaccid  para- 
lysis with  abolition  of  the  deep  reflexes  and  the  reaction  of 
degeneration  in  the  muscles  supplied  by  the  affected  roots. 
The  sensory  symptoms  are :  subjective  numbness,  tingling 
and  pain  referred  to  the  sensory  areas  supplied  by  the  affected 
roots.  The  sensory  loss  develops  later  and  is  of  the  peripheral 
type — a  point  of  the  utmost  importance  in  the  differential 
diagnosis  of  cauda  equina  from  intramedullary  lesions.     In 


Fig.  44. — The  three  figures  represent,  from  left  to  right,  the  successive  stages 
of  involvement  of  the  roots  of  the  cauda  equina  in  a  case  of  sarcoma. 
The  black  areas  are  those  of  complete  sensory  loss,  the  shaded  those  of 
incomplete  loss.     Compare  -with  fig.  91. 


root  lesions  the  protopathic  and  epicritic  areas  coincide,  the 
former  sometimes  overlapping  the  latter.  The  sphincter 
symptoms  are  slight  impairment  of  vesical  control  and 
constipation  if  the  sacral  roots  on  one  side  only  are  affected ; 
if  the  lesion  is  bilateral,  complete  loss  of  both  the  vesical  and 
anal  sphincters  is  present,  with  patulous  anus  and  loss  of 
sensation.  The  sensibility  of  the  genitalia  is  abolished  and 
erection  is  impossible. 

Two  distinct  types  are  met  with ;  the  first,  where  the  lesion 


122 


NERVOUS  DISEASES 


is  mesial  and  low  down,  and  the  second  where  it  is  peripheral 

and  towards  the  upper  part  of  the  cauda. 

1.  Symptoms  of  a  lesion  situated  mesially  and  low  down 

in  tJie  cauda  equina. 

The  earliest    symptoms  are  referred  to  the  lowest  sacral 

roots,  objective  sensory  loss  being  detected  around  the  anus, 

upon  the  genital  organs  and  over  the  buttocks.     This  is  at 

first  limited  to,  or  is  prepon- 
deratingly  on  one  side,  but 
eventually  extends  so  as  to 
affect  both  sides.  The 
earliest  bladder  and  rectal 
symptoms  consist  mainly 
of  a  loss  of  voluntary  con- 
trol, whereby  a  sudden 
evacuation  of  either  the 
bladder  or  the  rectum  may 
occur.  A  loss  of  sexual 
power  and  of  erection  are 
coexistent  conditions.  When 
the  lesion  is  definitely 
bilateral,  more  pronounced 
vesical  and  rectal  symptoms 
develop.  These  consist  of 
a  dribbling  of  urine  and 
incontinence  of  faeces  from 
paralysis  of  the  sphincters, 
the  patient  being  unaware  of 
the  passage  of  the  excreta. 

The  extension  of  the  lesion 
is  noted  by  a  progressive 
implication  of  the  third, 
second,  and  first  sacral,  and 
the  fifth  and  fourth  lumbar 
roots,  with  corresponding 
sensory  changes  over  these 
root  areas ;  motor  paralysis  with  atrophy  of  muscles  and 
loss  of  the  reflexes — first  the  plantar,  then  the  ankle  jerk, 
and  later  the  knee  jerk.     (Figs.  44  and  45.) 

In   this  type  therefore  the  vesical  and  genital    symptoms 
are  early,  the  motor  late  in  appearing. 


Fig.  45. — Photograph  showing  the 
escape  of  the  5th,  4th,  3rd,  and 
2nd  sacral  areas  in  a  case  of 
lesion  of  the  12th  dorsal  to  the 
1st  sacral  roots  on  the  right   side. 


THE  PERIPHERAL  NERVOUS  SYSTEM  L23 

2.  The  symptoms  of  a  lesion  situated  peripherally  and 
high  up  in  the  cauda  equina. 

This  type  is  usually  seen  in  association  with  meningeal 
growths  and  tumours. 

Pain  is  commonly  an  early  symptom  and  is  referred  to 
the  third,  fourth,  and  fifth  lumbar  or  the  upper  sacral 
roots.  Pain  remains  unilateral  for  a  considerable  time  ;  but 
eventually  objective  sensory  loss  is  detected  and  is  soon 
followed  by  motor  paralysis,  while  the  bladder,  rectal  and 
generative  functions  remain  intact.  Thus,  in  one  case  of 
gummatous  meningitis  the  earliest  symptoms  were  unilateral 
loss  of  sensation  over  the  third,  fourth,  and  fifth  lumbar  areas 
with  weakness  and  atrophy  of  the  corresponding  muscles,  loss 
of  the  knee  jerk  with  preservation  of  the  ankle  jerk  and  flexor 
plantar  response,  and  intact  bladder  and  rectal  functions. 
As  the  disease  extended,  the  first,  second,  and  third  sacral 
areas  became  involved  with  loss  of  the  ankle  jerk  and  plantar 
reflex,  and  symptoms  of  involvement  of  the  third  and 
fourth  lumbar  roots  upon  the  opposite  side.  Eventually 
the  whole  of  the  cauda  equina  on  both  sides  became 
affected,  sphincter  trouble,  bedsores,  and  death  eventually 
supervening. 

For  the  motor  and  sensory  localisation  of  the  several 
root  lesions,  the  reader  is  referred  to  the  Table  on  p.  317 
and  figs.  90  and  91. 

The  diagnosis  of  lesions  of  the  cauda  equina  has  to  be 
made  from  several  conditions. 

1.  Lesions  of  the  conus  medullaris  may  be  either  prim- 
ary, or  occur  in  association  with  lesion  of  the  cauda  equina. 
The  symptoms  which  characterise  a  lesion  limited  to  the 
conus  are,  loss  of  control  over  the  bladder  and  rectum — 
dribbling  of  urine,  and  either  constipation  or  involuntary 
evacuation  of  the  rectum ;  loss  of  sensation  (total  or  dissociated) 
over  the  ano-perineeal  region  and  genitalia — testicular  tender- 
ness being  preserved  ;  loss  of  voluntary  or  reflex  sexual  action  ; 
and  anaesthesia  over  the  areas  on  the  buttocks  supplied  by  the 
third  and  fourth  sacral  roots.  There  is  no  motor  disability, 
and  the  deep  and  superficial  reflexes  are  normal.  The  onset 
and  course  of  the  symptoms  are  more  rapid  than  in  lesions  of 
the  cauda  and  are  more  often  bilateral.  The  dissociation  of 
sensation  is  often  striking,  and  may  serve  to  distinguish  a 


124  NERVOUS  DISEASES 

lesion  of  the  conns  from  one  situated  mesially  and  low  down 
in  the  -cauda. 

If  the  lesion  in  the  conns  extends  upwards  so  as  to  involve 
the  first  and  second  sacral  segments,  loss  of  sensation  over 
the  soles  of  the  feet  and  over  a  strip  of  skin  up  the  centre 
of  the  posterior  surface  of  the  lower  limhs  is  found.  This 
is  associated  with  paralysis  of  the  glutei,  triceps,  semi-mem- 
hranosus,  tibialis  posticus  and  calf  muscles,  and  the  intrinsic 
muscles  of  the  feet.  The  plantar  reflexes  and  ankle  jerks 
are  impaired  or  abolished. 

2.  A  lesion  of  the  spinal  cord  at  the  level  of  the  second  and 
third  lumbar  segments  may  resemble  one  of  the  cauda  equina. 
The  differentiation  is  difficult  only  if  the  lesion  is  total,  when 
there  would  be  found  complete  atrophic  flaccid  paralysis  of 
the  lower  limbs,  complete  loss  of  all  forms  of  sensation, 
abolition  of  the  reflexes  and  total  sphincter  paralysis.  This 
association  of  symptoms  could  only  be  attributed  to  the  cauda 
equina  as  a  result  of  a  traumatic  lesion.  In  the  absence  of 
this  histoi'37  it  would  have  to  be  ascribed  to  a  total  transverse 
lesion  of  the  cord. 

3.  Malignant  and  tuberculous  disease  of  the  sacrum 
may  give  rise  to  symptoms  of  lesions  of  the  cauda  equina. 
These  diseases  are  of  slow  and  gradual  onset,  with  ill -defined 
though  sometimes  sharp  and  shooting  pains  in  the  sacral 
region.  Pain  is  usually  present,  often  more  intense  in  the 
recumbent  posture. 

The  nerve  symptoms  depend  upon  the  implication  of  the 
nerve  roots,  which  may  be  individually  picked  out  by  the 
progressive  extension  of  the  maladj7.  The  local  objective 
signs  are  slight  in  contrast  to  the  severity  of  the  subjec- 
tive symptoms  and  the  obvious  cachectic  condition  of  the 
patient.  Local  prominence  and  tenderness  on  pressure 
over  the  sacrum  should  be  looked  for,  and  in  all  cases  a 
careful  rectal  and  sigmoidoscopic  examination  should  be  made. 
The  Rontgen  rays  may  also  be  used  with  advantage  in  solving 
the  problem  of  the  diagnosis,  which  i3  often  for  a  time 
obscure. 

Lesions  op  the  Lumbo-Sackal  Plexus 

The  lumbo-sacral  plexus  is  a  complex  arrangement  of 
nerves,  and  may  be  divided  into  three  sub-systems:  the  lumbar, 


THE  PERIPHERAL  NERVOUS  SYSTKU 


125 


the  sacral  or  sciatic,  and  the  pudendal  plexuses.  The  lum- 
bar plexus  is  formed  by  the  anterior  branches  of  the  nerves 
issuing  from  the  first  to  the  fourth  lumbar  segments,  the 
sacral  or  sciatic  plexus  by  those  from  the  fifth  lumbar  to  the 


Genito-crural.  ~ 

Ilio-hypogastric.  — 

Ilio-inguinal.  — 

External  cutaneous. .- 

Obturator. .. 

Accessory  obturator.  .. 

To  iliacus  and  psoas.    ■ 

Anterior  crural.    ■ 


Superior  gluteal 

Inferior  gluteal. 

To  pyriformis 

Great  sciatic 

Quadratus  fern.   -yW'  '/^  •' ~'/  ef 


Ext.  popliteal.   --^SjKttiS 
Int.  popliteal. 


...  Firsi  Iwmbar. 
...  Second  lv/mbar. 

—  Third  lumbar. 
...Fourth  lumbar. 

.-.Fifth  lumbar. 
.  _  First  sacral. 

. .  Second  sacral. 

Third  sacral. 

Visceral  branches.\ 
.  Fourth  sacral. 
.  Visceral  branches.  |  I 

Perineal. 

.  Fifth  sacral. 
--  To  coccygeus. 
-To  levator  ani. 

-  Coccygeal. 


To  hamstrings 


Pudic. 
Small  Perforating 

obturator    sciatic-  cutaneous, 

internus. 


Ano-coccygeal. 


Fig.  46. — Diagram  of  a  common  form  of  mmbo-sacral  plexus. 
(From  Morris's  'Anatomy.') 


third  sacral,  and  the  pudendal  plexus  by  those  from  the  fourth 
and  fifth  sacral  and  coccj^geal  segments.    (Fig.  46.) 

The  causes  of  paralysis  of  the  nerves  of  the  lower  limb 
are  found  in  connexion    with    disease   of   the    bones   of   the 


126  NERVOUS  DISEASES 

lumbar  and  sacral  vertebra?,  and  of  the  pelvic  organs.  These 
nerves-  are  also  liable  to  injury  from  the  use  of  the  forceps 
during  delivery. 

The  great  sciatic  nerve  is  more  often  wounded  in  gunshot 
injury  than  any  other  nerve  of  the  body.  It  may  also  be 
paralysed  in  the  manipulations  of  reducing  congenital  dislo- 
cation of  the  hip. 

Various  toxic  conditions  may  also  lead  to  paralysis  of  the 
nerves  of  the  lower  limb.  Their  influence  is  seen  in  the 
alcoholic  and  arsenical  forms  of  peripheral  neuritis.  Puerperal 
neuritis  and  neuritis  following  upon  septic  conditions  within 
the  pelvis  have  also  been  observed.  Paralysis  of  the  external 
popliteal  nerve  is  an  early  symptom  of  diabetic  neuritis. 

It  is  a  well-known  fact  that  the  peroneal,  or  external 
popliteal  nerve  is  more  liable  to  paralysis  than  the  internal 
branch  of  the  great  sciatic. 

The  lumbar  plexus 

The  first  lumbar  nerve  gives  origin  to  the  ilio-hypogastric 
and  ilio-inguinal  nerves,  and  partly  to  the  genito- crural. 
These  nerves  are  mainly  sensory,  but  give  motor  filaments 
to  the  recti,  the  obliqui  abdominales,  and  the  cremaster 
muscles. 

The  chief  motor  branches  of  the  lumbar  plexus  arise  from 
the  second,  third,  and  fourth  lumbar  nerves.  They  are  the 
anterior  crural,  obturator,  and  external  cutaneous  nerves. 

The  anterior  crural  nerve 

The  anterior  crural  nerve  supplies  the  quadriceps  extensor, 
the  pectineus  in  part,  and  the  sartorius  muscles.  Common 
causes  of  paralysis  of  this  nerve  are  tumours  of  the  pelvis  and 
psoas  abscess ;  a  primary  neuritis  may  sometimes  affect  it. 
Its  paralysis  is  characterised  by  inability  to  extend  the  knee 
joint,  and  by  abolition  of  the  knee  jerk.  The  quadriceps 
muscle  is  wasted. 

There  is  loss  of  epicritic  and  protopathic  sensation  over  an 
area  situated  on  the  inner  aspect  of  the  leg  extending  from 
the  inner  and  upper  border  of  the  plantar  arch,  over  the 
internal  malleolus   to  more   than  halfway   up  the   leg.     Its 


THE  PERIPHERAL  NERVOUS  SYSTEM 


127 


upper  border  becomes  less  distinct  and  merges  into  a  large 
area  over  the  front  and  inner  aspect  of  the  thigh,  in  which 
there  is  no  complete  loss  of  sensibility  (Sherren).     (Fig.  47.) 


The  obturator  nerve 

The  obturator  nerve  supplies  the  adductors  of  the  thigh,  the 
obturator  externus,  the  pectineus  (partly),  and  the  gracilis 
muscles.  Hence  its  paralysis  impairs 
the  approximation  of  the  thighs,  outward 
rotation  of  the  hip  joint,  and  the  move- 
ment of  crossing  the  legs.  There  is 
wasting  on  the  inner  aspect  of  the  thigh. 
It  may  be  injured  in  prolonged  instru- 
mental delivery. 

There  is  no  definite  sensory  loss. 

The  external  cutaneous  nerve 

The  external  cutaneous  is  a  sensory 
nerve,  and  supplies  the  skin  over  the 
outer  aspect  of  the  thigh  with  epicritic 
and  protopathic  sensation. 

A  condition — meralgia  paraesthetica — 
characterised  by  paresthesia,  and  accom- 
panied by  more  or  less  objective  distur- 
bance of  the  sensibilities  of  touch,  pain, 
and  temperature,  has  been  described 
over  the  distribution  of  the  nerve  on  the 
outer  side  of  the  thigh.  There  may  be 
tenderness  on  pressure  over  the  nerve  at  the  point  where 
it  issues  from  the  deep  fascia.  In  a  case  of  this  affection 
E.  Bramwell l  found  no  sign  of  neuritis  in  a  portion  of 
the  nerve  which  had  been  excised. 


Fig.  47.— The  area  of 
sensory  loss  from 
a  lesion  of  the  an- 
terior crural  and 
its  internal  saphe- 
nous branch. 

(After  Head.) 


The  Saceal  Plexus 

The  sacral  or  sciatic  plexus  is  formed  by  the  nerves  from 
the  fifth  lumbar  to  the  third  sacral,  including  a  branch 
from  the  fourth  lumbar  nerve.     It  gives  origin  to  one  large 

1  Bramwell  (Edwin),  Edin.  Med.  Journal,  1903. 


L28 


NERVOUS  DISEASES 


composite  nerve  trunk — the  great  sciatic  nerve.  This  is  formed 
by  two  separate  and  distinct  nerves  usually  contained  in  one 
sheath  —  the  internal  popliteal  or  tibial  nerve,  formed  by 
the  anterior  branches  of  the  plexus,  and  the  external  popliteal 
or  peroneal  nerve,  formed  b}r  the  posterior  branches. 


Fi<;.  48. — Front,  back,  and  side  views  of  the  leg  stowing  the  distribution  of 
the  loss  of  sensibility  following  injury  to  the  great  sciatic  nerve.  The 
black  area  represents  the  zone  of  protopathic  and  epicritic  loss,  the 
continuous  line  the  extent  of  the  epicritic  overlap.      (After  Head.) 

In  addition  to  the  great  sciatic  nerve,  several  subsidiary 
nerves  are  described  as  arising  from  the  sciatic  plexus :  (a) 
the  nerve  to  the  tensor  fasciae  femoris  from  the  fourth  lumbar  ; 
{b)  the  superior  gluteal  nerve  to  the  gluteus  medius  and  mini- 
mus, from  the  fourth  and  fifth  lumbar  and  first  sacral ;  (c)  the 
inferior  gluteal  nerve  to  the  gluteus  maximus,  from  the  fifth 
lumbar  and  first  and  second  sacral ;  (d)  branches  from  the 
first  three  sacral  nerves  which  pass  downwards  to  join  the 
pudendal  plexus  and  form  the  small  sciatic  nerve. 


The  great  sciatic  nerve 

Paralysis  of  all  the  muscles  supplied  by  both  divisions  of 
the  nerve  is  theoretically  possible,  but  rarely  occurs.  More 
commonly  there  is  paralysis  of  one  or  other  of  its  branches, 


THE  PERIPHERAL  NERVOUS  SYSTEM  129 

in  whole  or  in  part,  and  almost  invariably  the  external 
popliteal  branch  is  that  which  suffers  most. 

If  the  lesion  is  high  up  in  the  thigh  the  motor  symptoms 
consist  of  paralysis  of  all  the  muscles  below  the  knee,  the 
flexors  of  the  knee,  and  the  flexor  portion  of  the  adductor 
magnus.  The  sensory  loss  is  of  wide  distribution  and  con- 
sists of  epicritic  and  protopathic  anaesthesia  below  the  knee, 
except  for  a  strip  on  the  inner  aspect  of  the  leg,  which 
corresponds  to  the  supply  of  the  internal  saphenous  nerve. 
(Fig.  48.) 

The  nerve  to  the  hamstrings  arises  from  all  the  roots 
forming  the  tibial,  or  internal  popliteal  division  of  the  great 
sciatic  (L  4  and  5,  S  1,  2  and  8).  Although  passing  along  with 
the  sciatic  in  the  upper  part  of  its  course,  it  is  given  off  high 
up  in  the  thigh,  and  thus  escapes  gunshot  and  other  wounds 
of  the  sciatic  in  this  region.  The  absence  of  paralysis  of 
the  hamstring  muscles  (biceps,  semi-membranosus  and 
semi-tendinosus),  with  palsy  of  the  tibial  nerve,  is  therefore 
significant  of  a  lesion  of  the  sciatic  in  the  lower  half  of 
the  thigh,  while  a  coexistent  palsy  of  the  hamstrings,  the 
calf  muscles,  and  the  flexors  of  the  toes,  points  to  a  lesion 
either  of  the  sciatic  plexus,  the  cauda  equina,  or  the  spinal 
segments. 


The  internal  popliteal  nerve 

Lesion  of  the  internal  popliteal,  or  tibial,  nerve  is  followed 
by  paralysis  of  the  muscles  of  the  calf  of  the  leg,  the  tibialis 
posticus,  and  the  long  flexors  of  the  toes  and  hallux.  Through 
its  internal,  or  plantar  branch — which  corresponds  to  the 
median  nerve  in  the  hand — it  supplies  the  flexor  brevis 
digitorum,  the  small  muscles  of  the  big  toe  (except  the 
adductor  hallucis),  and  the  two  inner  lumbricals.  Through 
the  external  plantar  branch — which  is  analogous  to  the 
ulnar  nerve — it  supplies  the  muscles  of  the  little  toe, 
the  two  outer  lumbricals,  the  adductor  hallucis,  and  all  the 
interossei. 

In  paralysis  of  the  nerve  the  foot  and  toes  cannot  be 
pointed,  nor  the  toes  flexed  ;  and  inversion  of  the  foot  in  the 
extended  position  is  impossible.    The  foot  assumes  the  position 

9 


130 


NERVOUS  DISEASES 


of  talipes  calcaneo-valgus.  The  toes  are  lryper-extended  at 
the  proximal,  and  flexed  at  the  distal  joints. 

In  severe  lesions  hoth  epicritic  and  protopathic  sensibilities 
are  lost  over  the  sole  of  the  foot,  and  along  its  outer  border,  as 
far  up  as  the  outer  ankle  bone.     (Fig.  49.) 

In  less  severe  cases,  epicritic  sensibility  alone  is  impaired 
or  lost.     In  one  case  of  this  character  the  area  of  epicritic 


Fig.  49. — Areas  of  protopathic  and  epicritic  loss  from  lesion  of  the  posterior 
tibial  nex*re.     The  shading  is  the  same  as  for  fig.  39.     (After  Head.) 

loss  covered  the  sole  of  the  foot  and  toes,  the  outer  border 
and  part  of  the  dorsum  of  the  foot  as  far  as  the  external 
malleolus,  and  the  dorsal  aspect  of  the  distal  phalanges  of 
the  toes. 

Deep  sensibility  is  unaffected. 


The  external  popliteal  nerve 

The  external  popliteal  or  peroneal  nerve  supplies  the 
tibialis  anticus,  extensor  longus  digitorum,  extensor  proprius 
hallucis,  the  peroneal  muscles,  and  the  extensor  brevis 
digitorum. 

In  paralysis  of  the  nerve,  the  foot  cannot  be  dorsiflexed  or 
everted,  nor  can  the  toes  be  extended.  The  characteristic 
appearance  of  drop   foot  is   seen  with    a  tendency  towards 


THE  PERIPHERAL  NERVOUS  SYSTKM 


131 


talipes  equino-varus,  and  later  flexor  contracture  of  the  toes. 
In  lesions  high  up  there  is  loss  of  epicritic  and  protopathic 
sensibility  over  the  outer  aspect  of  the  leg  from  below  the 
knee  and  extending  on  the  dorsum  of  the  foot  to  the  metatarso- 
phalangeal joints  of  the  toes.  In  lesions  below  the  level  of 
origin  of  the  lateral  cutaneous  branch,  the  sensory  loss  is  over 
the  dorsum  of  the  foot,  and  only  extends  up  the  outer  aspect 


Fig.  50. — Areas  of  protopathic  and  epicritic  loss  after  lesion  of  the  external 
popliteal  nerve.     The  shading  is  the  same  as  in  fig.  39.    (After  Head.) 


of  the   leg  for  a   few   inches  above   the  external   malleolus 
(fig.  50).     (Sherren.) 


The  Pudendal  Plexus 

This  plexus  is  formed  partly  by  the  small  sciatic  nerve, 
which  arises  from  the  first  to  the  third  sacral  nerves,  and 
partly  by  the  fourth  and  fifth  sacral  and  the  coccygeal  nerves. 
It  is  distributed  to  the  skin  over  the  back  of  the  thigh  and  the 
genital  organs.  It  supplies  the  perineal  muscles,  levator  ani, 
external  sphincter  ani,  and  erector  penis. 

Owing  to  its  position  in  the  lower  and  back  part  of  the 
pelvis,  this  plexus  is  rarely  the  seat  of  disease  or  of  traumatism. 
Paralytic  symptoms  over  its  area  of  distribution  are  usually 
due  to  lesion  of  the  sacral  nerve  roots  or  the  cauda  equina. 

9* 


132  NERVOUS  DISEASES 

Chaptbk  IV 

TUMOURS  OF  NBRYES 

New  growths  may  involve  the  roots  and  the  peripheral 
nerves  in  any  part  of  their  course.  They  may  be  single 
or  multiple,  benign  or  malignant.  They  may  be  classified 
as  follows : — 

1.  True  neuromata. 

2.  False  neuromata. 

(a)  Benign  :    fibromata,    myxomata,    lipomata,    fibro- 

nryxoinata,  and  cystic  tumours.     Such  tumours 
may  be  solitary  or  multiple. 

(b)  Malignant :  sarcomata  and  carcinomata. 

(c)  Amputation  neuromata. 

True  neuromata  are  rare,  and  are  only  found  in  con- 
nexion with  the  sympathetic  nervous  system — especially  in 
the  abdominal  plexuses  and  in  the  cellular  tissues.  They  ma}7 
be  single  or  multiple,  circumscribed  or  diffused.  They  are 
innocent  in  character.  Their  size  varies  from  that  of  a  pea 
to  that  of  a  grape-fruit. 

They  are  often  present  in  children,  but  may  not  develop 
till  later,  when  the  sympathetic  system  is  fully  formed.  The 
tumours  are  of  a  firm  or  elastic  consistence,  and  are  encapsu- 
lated. Microscopically  they  are  composed  of  sympathetic 
ganglion  cells  and  non-medullated  nerve  fibres,  with  an 
abundant  supply  of  blood-vessels.  The  nerve  cells  are  round 
or  oval  in  shape,  unipolar  with  clear  or  granular  protoplasm, 
and  contain  a  distinct  nucleus  or  nucleolus. 

Clinically  there  are  no  nervous  symptoms,  and  the 
tumours  are  not  tender  on  pressure. 

False  neuromata.  Under  this  term  are  classed  all  the 
benign  and  malignant  tumours  occurring  in  association  with 
the  peripheral  nervous  system,  as  well  as  the  bulbous  tumours 
found  after  injury  or  division  of  nerves. 

The  benign  tumours  are  fibromata,  myxomata,  lipomata, 
fibro-myxomata,  and  cystic  tumours,  resulting  from  degenera- 
tion. They  may  be  single  or  multiple.  The  solitary  tumours, 
which  have  the  histological  characters  of  those  already  men- 
tioned, may  occur  in  any  position,  either  on  the  roots,  trunks, 


THE  PERIPHERAL  NERVOUS  SYSTEM  133 

or  subcutaneous  filaments  of  the  nerves.  The  common 
localities  for  these  tumours  are  the  auditory  nerve,  where 
they  form  one  of  the  varieties  of  extra-cerebellar  growth 
(p.  267)  ;  the  fifth  cranial  nerve,  and  the  posterior  nerve 
roots  within  the  spinal  canal,  where  they  form  one  of 
the  causes  of  compression  paraplegia  (p.  348).  They  may 
be  both  intra-  and  extradural  in  situation.  They  are  also 
not  uncommon  on  the  nerve  trunks,  but  are  more  frequently 
seen  upon  the  nerve  filaments,  where  they  form  painful 
subcutaneous  tubercles. 

The  benign  growths  are  found  in  persons  otherwise  healthy. 
They  usually  come  on  between  the  twentieth  and  fortieth 
years,  and   affect  women  slightly  more  often  than  men. 

Symptoms.  The  symptoms  of  intracranial  and  intra- 
neural growths  are  elsewhere  described  (pp.  267  and  348). 

When  situated  on  a  sensory  nerve,  they  give  rise  to 
neuralgic  pains.  When  upon  a  mixed  nerve,  the  pain  may 
be  less  severe,  and  is  associated  with  motor  weakness. 
Complete  sensory  and  motor  paralyses  are  practically 
unknown.  The  pain  may  be  located  at  the  seat  of  the 
tumour,  or  referred  to  the  course  of  the  nerve.  It  may  be 
constant  with  exacerbations,  or  may  be  intermittent. 

On  examination,  a  swelling  of  varying  consistence  may 
be  felt  in  the  course  of  the  nerve.  The  tumour  is  painful  on 
pressure,  and  its  recognition  is  usually  easy.  In  some  cases 
its  presence  has  been  observed  by  the  patient  for  some  time 
before  the  onset  of  symptoms.  If  a  deep  nerve  is  the  seat  of 
a  growth,  the  diagnosis  is  more  difficult,  but  the  distribution 
of  the  pain  and  motor  weakness,  taken  in  association  with  the 
finding  of  a  localised  tender  spot  on  pressure  somewhere  in 
the  course  of  the  nerve,  is  significant  of  the  condition. 

Fibromata  are  of  slow  growth,  usually  circumscribed  and 
encapsulated,  and  composed  of  fibrous  tissue  arranged  in 
whorls ;  the  blood-vessels  are  well  developed,  and  the 
growth  does  not  invade  the  nerve  fibres,  which  merely  suffer 
by  pressure. 

Multiple  benign  growths  are  of  various  types  : — 

(a)  Multiple  neuro  fibromata  or  general  fibromatosis. 
In  this  condition  the  nerves  present  an  irregular,  diffuse, 
thickened  appearance,  and  may  be  the  seat  of  multiple  tumours 
similar    to   those    described  under  the  solitary  forms.     The 


134  NERVOUS  DISEASES 

essential  character  of  this  condition  is  an  increase  of  the 
fibrous  tissue  of  the  endoneurium  with  general  thickening 
of  the  epineurium.  In  some  cases  the  nerve  fibres  are 
degenerated.  The  posterior  nerve  roots  and  ganglia  may 
be  affected  as  well  as  the  peripheral  nerves. 

Within  the  neural  canal,  the  cauda  equina  and  cervical 
roots  are  most  frequently  involved.  Any  of  the  cranial 
nerves  but  especially  the  fifth,  seventh,  eighth,  and  tenth- 
may  be  involved. 

Clinically,  in  striking  contrast  to  the  almost  invariable 
tenderness  of  the  single  tumours  of  nerves,  general  neuro- 
fibromatosis is  characterised  by  an  absence  of  tenderness 
on  pressure  ;  but  during  the  course  of  the  disease,  one 
or  more  of  the  tumours  may  give  rise  to  pain  and  local 
tenderness. 

(b)  Plexiform  neuromata.  In  this  case  the  change  is 
confined  to  one  or  more  contiguous  nerves  or  plexuses. 
Pathologically,  this  variety  is  similar  to  that  just  described. 
Its  most  common  situations  are  the  forehead  and  temple, 
the  posterior  part  of  the  neck,  and  the  extremities. 

(c)  Cutaneous  neurofibromata — Molluscum  fibrosum,  or 
von  Recklinghausen's  disease.  The  distribution  of  this 
affection  is  generalised  over  the  whole  body,  with  the  exception 
of  the  palms  and  the  soles.  The  tumours  are  mainly  of 
soft  consistence  and  subcutaneous. 

Malignant  tumours  are  sarcomatous  and  carcinomatous. 
The  sarcomata  are  often  primary,  or  may  develop  from 
a  simple  fibroma.  They  are  spindle  or  round-celled,  and 
may  be  single  or  multiple.  They  tend  to  invade  the  nerve 
and  destroy  the  nerve  fibres. 

The  symptoms  at  the  onset  are  similar  to  those  found 
in  benign  growths,  but  progress  more  rapidly,  and  the 
ultimate  paralytic  effects  are  more  severe.  The  growth 
tends  to  spread  along  the  nerves,  and  when  situated  in  the 
intraneural  or  intracranial  cavities,  involves  the  dura  mater 
and  adjacent  nerves. 

They  are  common  upon  the  cauda  equina  and  the  cranial 
nerves,  but  are  less  frequent  upon  the  peripheral  nerves  of 
the  limbs. 

The  carcinomata  are  rare. 

Amputation   neuromata    are    not  tumours   in   the    strict 


THE  PERIPHERAL  NERVOUS  SYSTEM  135 

sense,  but  are  rather  part  of  the  normal  regenerative  process, 
and  as  such  contain  newly  formed  nerve  fibres. 

Treatment.  In  cases  of  single  benign  tumours  of  nerves, 
removal  of  the  growth  is  satisfactorily  and  successfully  carried 
out  by  ordinary  surgical  methods  ;  but  in  general  neurofibro- 
matosis, when  pain  is  a  complicating  symptom,  it  is  preferable 
to  resect  a  portion  of  the  nerve  higher  up,  to  divide  the 
corresponding  posterior  roots,  or  to  amputate  the  affected 
limb. 

Syphilis  of  the  Peripheral  Nerve 

Syphilitic  affections  of  the  nerves  may  occur  in  combina- 
tion with  intracranial  syphilis,  but  are  much  more  commonly 
found  in  association  with  multiple  gummata  of  the  bones  and 
muscles.  The  changes  may  be :  (1)  involvement  of  the 
nerve  by  an  extra-neural  gumma,  (2)  gummata  in  connexion 
with  the  sheath  of  a  nerve,  (3)  a  thickening  of  the  nerve 
sheath  and  of  the  peri-  and  endoneurium,  and  (4)  occasionally 
and  in  association  with  thickening  of  the  nerve  sheath 
obstruction  of  the  vasa  nervorum,  and  parenchymatous 
degeneration  of  the  myeline  sheath  from  arrest  of  the  blood 
supply. 


Chapter  V 
MULTIPLE    NEURITIS 

(Syn.  :  Peripheral  Neuritis,  Polyneuritis) 

Multiple  neuritis  is  a  disease  affecting,  more  or  less 
simultaneously  and  symmetrically,  all  or  many  of  the 
peripheral  nerves. 

Pathology.  The  pathological  changes  found  in  multiple 
neuritis  are  of  two  kinds  :  (a)  interstitial  neuritis,  characterised 
by  inflammation  and  proliferation  of  the  nerve  sheath  and 
fibrous  tissues  of  the  peri-  and  endoneurium  ;  (b)  parenchy- 
matous neuritis  shown  by  degeneration  and  disintegration  of 
the  medullary  sheaths  and  destruction  of  the  axis  cylinders. 

These  changes  are  most  intense  towards  the  termination 


136  NERVOUS  DISEASES 

of  the  nerves,  and  may  be  followed  by  secondary  alterations  in 
the  cells  of  the  anterior  horns  and  in  the  muscles. 

In  certain  cases  the  primary  changes  are  limited  to  the 
fibrous  structures,  the  parenchymatous  changes  being  secon- 
dary and  slight.  In  others,  the  lesion  is  confined  to  the 
anterior  horn  cells  and  the  nerve  fibres.  In  most  cases, 
however,  both  types  are  combined.  These  differences  would 
seem  to  depend  upon  the  selective  action  of  the  toxic  agent, 
but  in  both  varieties  the  peripheral  portions  of  the  nerves 
suffer  more  intensely  than  the  proximal. 

Varieties.  The  following  etiological  classification  embraces 
all  the  well-recognised  forms  of  multiple  neuritis: 

1.  Cases  due  to  poisons  derived  from  outside  the  body — 
alcohol,  the  coal-tar  products,  lead,  arsenic,  mercury,  copper, 
and  phosphorus. 

2.  Cases  due  to  poisons  acquired  or  developed  within  the 
body  as  the  result  of  infective  disorders— diphtheria,  influenza, 
malaria,  typhoid  fever,  scarlatina,  puerperal  fever,  gonorrhoea, 
septicaemia,  and  beriberi. 

3.  Cases  associated  with  general  disorders — diabetes,  gout, 
rheumatism,  anaemia,  malnutrition,  tubercle,  and  carcinoma. 

4.  Cases  due  to  the  local  action  of  organisms — leprosy 
and  syphilis. 

The  commonest  form  of  multiple  neuritis  in  this  country 
is  that  due  to  alcohol.  It  may  therefore  be  taken  as  the 
t}rpe  of  the  disease,  and  will  be  described  in  some  detail. 


ALCOHOLIC    NEURITIS 

Etiology.  Alcoholic  paralysis  occurs  more  frequently  in 
women  than  in  men,  a  fact  which  may  be  accounted  for  by 
the  lesser  resistance  of  the  female  sex  to  the  effects  of  alcohol, 
and  by  the  greater  incidence  of  secret  and  persistent 
drinking  amongst  women.      It  is  a  disease  of  adult  life. 

It  is  a  well-recognised  fact  that  the  disease  is  found  not 
so  much  in  those  who  drink  openly  as  in  those  who  drink  in 
secret.  It  is  also  more  common  in  soakers  than  in 
those  who  give  way  to  periodic  drinking  bouts,  and  affects 
spirit  more  than  beer  drinkers.  The  exciting  causes  are 
exposure  to  cold,  privation,  and  malnutrition. 


THE  PERIPHERAL  NERVOUS  SYSTEM 


137 


Symptomatology.  Alcoholic  neuritis  manifests  itself 
clinically  in  three  types — the  sensory,  the  motor,  and  the 
ataxic.  They  may  all  be  combined  in  the  same  case,  but 
one  type  usually  predominates  in  each  individual  instance. 

The  sensory  type.  The  earliest  subjective  symptoms  are 
numbness  or  deadness  affecting  first  the  feet  and  later  on  the 
hands.  This  is  rapidly  followed  by  the  occurrence  of  pain  on 
movements  of  the  limbs  and  by  painful  cramps,  especially  in 


Fig.  51. — Showing  the  characteristic  drop  feet  of  alcoholic  neuritis. 


the  legs.  Owing  to  these  troubles  the  patient  avoids  muscular 
stress  and  strain,  and  takes  to  lying  in  bed  or  in  an  easy 
chair,  so  as  to  maintain  the  muscles  in  a  state  of  relaxation. 
Trophic  changes  soon  show  themselves  in  the  skin  of  the 
extremities,  especially  of  the  hands  and  feet,  which  are  usually 
moist  and  of  a  sodden  appearance.  Some  loss  of  power  is 
also  observed  in  the  muscles  of  the  limbs  :  at  first  due  to 
an  unwillingness  to  move  the  muscles,  owing  to  the  pain  on 
movement  rather  than  to  any  actual  paralysis.  As  time  goes 
on  the  symptoms  increase,  and  contractures  take  place  in  the 
muscles.  In  the  lower  limbs,  the  hip  and  knee  joints  are 
flexed  and  the  foot  dropped  at  the  ankle.  In  the  upper  limbs, 
the  fingers  and  the  wrist  are  flexed,  owing  to  the  pre- 
dominant weakness  of  the  extensors.  Eventually  the  limbs 
become  more  or  less  fixed  in  these  positions. 

The  muscles   show   general  wasting,   though    not    to    any 


138  NERVOUS  DISEASES 

marked  extent,  and  the  skin  becomes  thin,  dry,  transparent, 
and  glossy.  The  hands  and  feet,  and  more  especially  the 
lingers  and  toes,  are  thinned  and  tapering.  Eventually  the 
patient  becomes  bedridden  and  powerless,  but  rarely  develops 
bedsores.  Often  a  well-marked  tremor  is  seen  in  the  hands, 
face,  and  lips. 

With  the  progress  of  the  disease  the  mental  state  shows 
increasing  general  enfeeblement.  The  patient  becomes 
garrulous  and  emotional,  crying  or  laughing  alternately,  and 
almost  invariably  exhibiting  a  piteous  craving  for  sympathy. 

Mental  symptoms.  It  is  noticed  that  persons  who  had 
previously  shown  a  clear  and  open  temperament  become 
secretive  and  cunning,  lose  their  finer  moral  feelings,  and 
exhibit  an  indifference  to  what  is  right  and  wrong.  This 
temperamental  change  may  be  accompanied  by  an  abnormal 
sensitiveness  to  questions  referring  to  the  use  or  abuse  of 
alcohol. 

A  further  obvious  impairment  of  the  mental  faculties  is 
characterised  by  loss  of  memory  for  recent  events,  mental 
inertia,  and  blunting  of  mental  acuteness  and  perception. 
Little  by  little  this  becomes  associated  with  irritability,  loss 
of  self-control,  and  emotional  instability.  In  addition  a  most 
characteristic  feature,  '  disorientation  '  or  loss  of  appreciation  of 
time  and  place,  is  noticed.  Eventually  such  patients  become 
dirty  and  careless  in  their  habits,  not  from  any  weakness  of 
sphincter  control,  but  from  loss  of  self-respect  and  want  of 
attention  to  personal  appearances.  In  some  cases  the  onset 
of  the  disease  is  preceded  by  an  attack  of  delirium  tremens. 

The  cranial  nerves  are  seldom  affected,  but  the  conjugate 
movements  of  the  eyes  are  easily  fatigued  and  not  well 
sustained.     Sometimes  nystagmoid  jerkings  are  observed. 

It  is  rare  to  find  peripheral  facial  palsy,  although  it 
occasionally  occurs.     The  tongue  is  tremulous. 

Motor  system.  Motor  weakness  is  earliest  seen  in  the 
extremities  of  the  limbs,  especially  in  the  actions  of  dorsiflexion 
of  the  feet  and  extension  of  the  wrist  and  fingers.  Wasting 
of  the  muscles  is  not  usually  marked,  but  electrical  stimulation 
may  reveal  a  partial  or  complete  reaction  of  degeneration. 

Sensory  system.  The  patient  complains  of  deadness, 
numbness,  tingling,  and  burning  in  the  hands  and  feet  and 
cramp-like  pains  in  the  muscles. 


THE  PERIPHERAL  NERVOUS  SYSTEM  139 

There  is  loss  or  impairment  of  all  forms  of  cutaneous 
sensibility,  especially  over  the  distal  portions  of  the  limbs 
with  a  shading  off  towards  the  proximal  parts. 

Superficial  tenderness  is  occasionally  present ;  but  in  all 
cases  there  is  a  great  muscular  hyperesthesia  and  even 
moderate  pressure  on  the  muscles  may  evoke  great  agony — a 
point  of  differential  diagnosis  from  tabes  dorsalis. 

The  sense  of  passive  position  and  of  movement  of  the 
limbs  is  impaired. 

Reflexes.  The  deep  reflexes  are  diminished  and  finally 
lost.  The  superficial  are  increased  at  first,  but  later  may 
become  diminished  or  absent. 

The  sphincters  are  not  affected ;  but  in  the  later  stages, 
owing  to  the  mental  condition,  the  calls  of  nature  are  not 
attended  to. 

Trophic.  The  skin  of  the  extremities  is  at  first  moist 
and  sodden,  but  later  shows  dryness  and  glossiness,  some- 
times shedding  fine  epithelial  scales,  which  appear  like  a  thin 
tissue  over  the  underlying  skin.  In  this  way  the  typical 
tapering,  thin,  dry,  glossy,  and  semi-translucent  hand  and 
fingers  are  produced.  An  cedematous  condition  of  the 
extremities  is  sometimes  found  in  cases  with  cardiac  and 
renal  complications.  The  nails  become  ribbed  and  brittle, 
and  the  hair  fine  and  thin.  Trophic  joint  changes  with 
adhesions  may  also  develop  and  cause  permanent  deformity, 
even  in  cases  which  recover. 

The  motor  type.  In  this  variety  of  alcoholic  neuritis  the 
earliest  symptoms  are  weakness  of  the  legs  and  the  readiness 
with  which  fatigue  is  induced.  The  weakness  is  at  first  seen 
in  the  distal  portions  of  the  extremities,  especially  in  the  dorsi- 
flexors  of  the  feet  and  the  extensors  of  the  wrist  and  fingers. 
The  toes  catch  the  ground  in  walking,  and  steppage  gait, 
drop  foot,  and  drop  wrist,  are  characteristic.  The  degree 
of  weakness  may  be  out  of  all  proportion  to  the  discernible 
muscular  atrophy,  although  in  some  cases  an  early  and  rapid 
muscular  wasting  occurs,  the  muscles  becoming  flabby,  hyper- 
esthetic,  and  losing  their  myotatic  irritability.  In  many 
the  wasting,  which  eventually  ensues,  does  not  appear  until 
a  late  stage. 

In  this  type  subjective  paresthesia  in  the  hands  and  feet 
is  almost  always  present,  as  well  as  muscular  hyperesthesia; 


140  NERVOUS  DISEASES 

but  cutaneous  sensibility  may  only  be  relatively  impaired. 
The  deep  reflexes  are  lost  early.  Trophic  changes  in  the  skin 
are  late  symptoms.  The  mental  condition  is  as  already 
described. 

The  ataxic  type.  In  this  type  inco-ordination  of  move- 
ment is  a  striking  feature,  and  may  come  on  with  the  earliest 
symptoms  of  motor  weakness.  It  is  most  marked  in  the 
legs,  and  is  always  associated  with  muscular  tenderness  and 
sometimes  with  muscular  hypotonicity.  Objective  cutaneous 
sensory  changes  are  minimal  in  degree  and  extent,  but  loss 
of  sense  of  position  of  the  limbs  is  relatively  more  pronounced. 

The  deep  reflexes  are  lost  early. 

This  form  of  peripheral  neuritis  is  probably  due  to  a 
proportionately  greater  affection  of  the  fibres  subserving 
deep  rather  than  epicritic  and  protopathic  sensibility. 

This  type  is  best  seen  in  the  subacute  and  chronic  cases 
of  the  disease. 

Complications.  Insomnia,  often  of  a  profound  and  trouble- 
some type ;  nausea,  retching,  sickness,  and  loss  of  appetite 
are  early  and  persistent  features.  Irregularity  of  the  heart's 
action,  often  associated  with  dilatation  and  fatty  changes, 
is  also  found. 

Bronchitis  in  older  subjects,  and  pulmonary  tuberculosis  in 
younger  patients,  constitute  grave  and  serious  complications. 

Albuminuria  is  frequent ;  but  the  most  serious  complica- 
tion is  the  liability  towards  pleural,  pericardial,  or  peritoneal 
effusions.  Febrile  disturbances  are  liable  to  produce  delirium 
and  hyperpyrexia,  from  which  recovery  is  rare. 

Prognosis.  If  the  case  is  seen  early  and  the  cause  can 
be  entirely  removed,  recovery  in  from  two,  three,  or  four 
months  may  take  place  under  appropriate  treatment.  In 
more  severe  cases,  recovery  may  also  be  obtained  as  a  result 
of  persistent  treatment  up  to  three  years.  In  cases  with 
much  mental  impairment,  recovery  is  never  complete,  as 
the  inertia  and  obstinacy  of  the  patient  militate  against 
all  attempts  at  improvement.  Second  and  third  attacks  are 
less  satisfactory,  and  recovery,  even  if  eventually  complete, 
may  be  delayed  for  months  or  years. 

Treatment.  The  first  essential  of  treatment  is  the  removal 
of  the  cause.  Simple  as  this  may  seem,  its  accomplishment 
is  impossible  unless  the  patient  is  removed  to  an  institution, 


THE  PERIPHERAL  NERVOUS  SYSTEM  141 

or  placed  under  the  direct  and  continuous  supervision  of 
trustworthy  attendants. 

Secondly,  the  patient  is  placed  in  bed  and  the  pain  eased, 
natural  sleep  induced,  and  the  disordered  digestion  corrected 
by  suitable  remedies  and  diet. 

Hypodermic  injections  of  strychnine  (3-5  minims  of  the 
liquor)  may  be  given  twice  or  thrice  daily,  combined  with 
general  tonic  treatment.  As  the  chief  hindrance  to  recovery 
is  the  weakness  of  will-power  of  the  patient  to  persevere  with 
treatment,  firmness,  patience,  and  encouragement  on  the  part 
of  the  physician  are  essential. 

Local  treatment.  One  of  the  earliest  and  most  important 
points  in  the  treatment  of  these  cases  is  to  prevent  the 
development  of  troublesome  contractures  of  the  hands  and 
feet.  This  is  effected  by  freeing  the  legs  from  the  weight  and 
pressure  of  the  bed-clothes  by  the  employment  of  a  cradle, 
and  by  seeing  that  the  limbs  are  maintained  and  supported 
in  a  correct  position  by  sand-bags  or  other  means. 

If  contractures  have  not  developed,  their  occurrence  is 
prevented  by  aid  of  gentle  passive  movements,  which  should  be 
employed  as  soon  as  possible.  If  already  present,  they  are 
overcome  by  the  adoption  of  mechanical  appliances — such  as 
1  Gowers's  boots,'  which  are  designed  to  counteract  the  over- 
action  of  the  calf  muscles  ;  and,  if  necessary,  of  the  flexors  of 
the  knees  by  the  aid  of  elastic  straps  attached  to  the  feet. 

Where  these  are  insufficient,  adhesions  and  contractures 
require  to  be  broken  down  under  anaesthesia,  and  extension 
afterwards  applied. 

As  soon  as  the  acute  stage  has  subsided,  galvanism  or 
galvano-faradisation  should  be  applied  to  the  limbs  in 
combination  with  gentle  massage,  in  order  to  maintain  and 
improve  the  condition  of  the  muscular,  sensory,  and  trophic 
systems.  Hot-air  baths  and  warm  local  applications  are  of 
service  both  in  the  relief  of  pain  and  in  assisting  the 
reparative  process  in  the  nerves. 

ARSENICAL    NEURITIS 

This  form  of  neuritis  occurs  in  those  who  are,  in  various 
ways,  subject  to  poisoning  by  arsenic  or  its  preparations.  Of 
these  may  be  mentioned  the  chronic  poisoning  which  occurs 


142  NERVOUS  DISEASES 

in  certain  industries  such  as  the  manufacture  of  wall  papers 
containing  arsenic  in  the  pigments,  in  sulphuric  acid 
factories,  and  in  the  manufacture  of  artificial  flowers.  A 
form  of  arsenical  neuritis  was  found  amongst  the  drinkers  of 
beer  brewed  from  glucose,  which  had  been  prepared  with 
contaminated  sulphuric  acid,  in  Manchester  in  1899  1900. 
It  has  also  been  seen  in  children  suffering  from  chorea 
who  have  been  treated  for  long  periods  with  Fowler's 
solution. 

Arsenic  affects  the  peripheral  nerves  by  producing  a  paren- 
chymatous neuritis  without  inflammation  of  the  interstitial 
tissues.  The  axis  cylinders  are  as  a  rule  preserved.  Arsenic, 
however,  like  lead,  has  an  effect  upon  the  nervous  system  as  a 
whole,  giving  rise  to  degenerative  changes  in  the  cells  of  the 
anterior  horns  of  the  cord  and  of  the  cortex  cerebri. 

The  symptoms  are  comparable  to  those  described  under 
alcoholic  neuritis.  The  earliest  symptoms  are  paresthesia 
and  pains  in  the  limbs,  to  which  paralysis  and  ataxia 
are  superadded.  There  is  usually  marked  loss  of  motor 
power  before  wasting  ensues.  The  reaction  of  degeneration 
is  found  in  the  muscles.  The  palsy  is  commonest  in  the  legs, 
is  bilateral  and  symmetrical,  and  affects  the  extensor  muscles 
in  excess  of  the  flexors.  The  result  is  drop  foot  and  drop 
wrist  often  of  a  marked  and  persistent  kind. 

Deformities  may  result  in  talipes  equino-varus  and  flexor 
contracture  at  the  wrist. 

In  some  cases  there  may  be  rapid  wasting  with  loss  of 
motor  power  coming  on  simultaneously,  but  in  these  cases 
spinal  cord  changes  probably  coexist. 

The  sensory  symptoms  are  excessive  hyperesthesia  of  the 
muscles  of  the  arms  and  legs,  and  objective  anesthesia  of  the 
peripheral  tpye. 

The  tendon  reflexes  are  abolished. 

Of  importance  in  the  diagnosis  of  arsenical  neuritis  are  the 
coexistent  skin  changes,  which  take  the  form  of  pigmentary 
discoloration  around  the  neck  and  on  the  abdomen.  The  skin 
is  dark  brown  and  mottled,  but  the  mucous  membranes  are 
never  discolored  as  in  Addison's  disease. 

Prognosis.  The  recovery  is  much  slower  than  in  the 
alcoholic  form,  and  even  after  recovery  from  the  paralysis, 
paresthesia  and  numbness  may  persist. 


THE  PERIPHERAL  NERVOUS  SYSTEM 


14.'5 


The  treatment  is  conducted  on  the  lines  laid  down  for 
alcoholic  neuritis. 

LEAD    NEURITIS 

Lead  neuritis  occurs  in  workers  in  lead — painters,  com- 
positors, and  plumbers — and  also  as  a  result  of  drinking 
water  contaminated  with  lead. 

Lead  also  exerts  extensive  general  effects  upon  the  nervous 
system :  first  by  its  direct  action  upon  the  central  nervous 
apparatus  (brain  and  spinal  cord),  and  secondly,  indirectly, 
through  its  action 
upon  the  blood- 
vessels, heart,  and 
kidneys.  In  conse- 
quence of  such  lesions 
it  may  give  rise  to 
numerous  disorders 
affecting  the  nervous 
system  other  than 
peripheral  neuritis 
— such  as  cerebral 
hemorrhage  and 
thrombosis. 

A  history  of  one  or 
more  attacks  of  lead 
poisoning — such  as  colic,  and  sometimes  of  a  previous  attack 
of  neuritis — is  generally  obtained.  The  patients  are  often 
anaemic,  and  in  almost  every  case  a  blue  line  may  be  observed 
along  the  margin  of  the  gums. 

The  symptoms  are  confined  to  the  motor  system,  sensory 
symptoms  being  very  rare.  A  fine  tremor  of  the  hands  is 
an  early  and  prominent  feature.  The  paralysis  affects  the 
arms  earlier  and  more  extensively  than  the  legs  :  in  some 
cases  being  limited  to  the  arms.  The  muscles  most  para- 
lysed are  the  extensors  of  the  wrist,  thumb,  and  fingers. 
(Fig.  52.)   _ 

The  distinction  between  a  musculo-spiral  paralysis  and  the 
multiple  form  of  peripheral  palsy  is  the  retention  of  the  power 
of  the  supinator  longus  muscle,  and  sometimes  of  the  extensor 
metacarpi  pollicis  in  the  latter  type. 


Fig.  52. — Showing  double  drop  wrist  in  a  case 
of  lead  neuritis. 


1  I  [  NERVOUS  DISEASES 

The  legs  are  only  rarely  affected,  in  which  event  the 
peronei  and  anterior  tibial  muscles  are  those  primarily 
paralysed. 

In  these  cases  wasting  and  muscular  atrophy  are  not 
marked,  although  the  reaction  of  degeneration  is  present ; 
sensory  changes  are  strikingly  absent. 

There  is  another  type  of  case  in  which  the  shoulder 
muscles  are  mainly  affected  :  the  deltoid  being  earliest 
involved,  and  later  the  biceps,  brachialis  anticus,  triceps,  and 
supinator  longus. 

On  the  other  hand,  a  type  has  been  described  in  which 
general  muscular  wasting — often  irregularly  distributed 
throughout  the  body,  but  sometimes  confined  to  the  small 
muscles  of  the  hands — is  associated  with  an  increase  of 
the  deep  reflexes  and  an  extensor  response.  These  cases, 
which  resemble  the  amyotrophic  type  of  palsy,  are  not  really 
neuritic,  but  are  due  to  an  associated  degeneration  of  the 
pyramidal  tract  and  anterior  horn  cells  of  the  spinal  cord. 

Prognosis.  The  disease  runs  a  long  course  of  several 
months,  recovery  taking  place  first  in  the  least  affected 
muscles ;  relapses  are  not  uncommon  on  returning  to  work. 

The  treatment  is  carried  out  on  the  general  lines  laid 
down  for  alcoholic  neuritis.  The  elimination  of  lead  from 
the  system  is  favoured  by  the  administration  internally  of 
iodide  of  potassium,  while  occasional  free  purgation  is  an 
important  aid. 

DIABETIC  NEURITIS 

It  has  long  been  recognised  that  peripheral  neuritis  may 
be  associated  with  the  presence  of  sugar  in  the  urine.  Its 
occurrence  is  probably  due  to  defective  metabolism  and 
auto-intoxication. 

Symptoms.  Diabetic  neuritis  differs  from  the  alcoholic  form 
in  that  subjective  pallesthesia  are  slight,  though  pain  may 
be  severe.  Almost  invariably  the  legs  are  more  affected 
than  the  arms,  and  the  external  popliteal  nerve  is  especially 
prone  to  suffer  in  the  early  stages.  Drop  foot  and  paralysis 
are  only  seen  in  the  severer  cases,  but  weakness  of  the  legs 
and  ataxia  may  be  present. 

The  ankle  jerks  are  abolished,  and  the  knee  jerks  may  be 


THE  PEKIPHERAL  NERVOUS  SYSTEM  145 

retained,  diminished,  or  lost.  Muscular  hyperesthesia  and 
slight  sensory  changes  are  present.  The  predominant  sensory 
change  is  loss  of  the  sense  of  vibration  (Williamson).1 

Trophic  sores,  either  in  the  form  of  gangrene  or  perfor- 
ating ulcer,  may  be  observed  in  the  feet,  but  are  distinguished 
from  the  tabetic  ulcers  by  the  fact  that  they  are  painful. 

We  have  observed  several  cases  in  which  there  was  for  a 
long  period  unilateral  isolated  affection  of  the  external 
popliteal  nerve. 

The  differential  diagnosis  of  diabetic  neuritis  from  tabes 
dorsalis  is  based  upon  the  absence  of  pupillary  changes, 
the  presence  of  muscular  hyperesthesia,  the  early  loss  of 
vibration  sensibility,  the  presence  of  slight  motor  weakness, 
and  by  the  detection  of  sugar  in  the  urine. 

The  treatment  of  this  condition  is  that  of  diabetes  mellitus 
(dietetic  and  medicinal)  combined  with  local  applications  of 
electricity  and  massage. 

POST-DIPHTHERITIC  NEURITIS 

Parenchymatous  neuritis,  with  occasional  interstitial 
changes,  is  the  characteristic  lesion  in  the  nerves  in  post- 
diphtheritic paralysis.  The  changes  are  best  seen  in  the 
intramuscular  portions  of  the  nerves ;  but  the  large  cells, 
both  of  the  anterior  horns  and  the  posterior  root  ganglia, 
may  be  affected. 

The  evidence,  so  far  as  ascertained,  seems  to  point  to  the 
anterior  horn  cells  of  the  spinal  cord  as  that  part  of  the 
nervous  system  which  is  first  affected.  This  is  followed  by 
degenerative  changes  in  the  most  peripheral  portions  of  the 
neurone,  and  later  on  in  the  whole  nerve. 

There  is  no  relation  between  the  severity  of  the  original 
disease  and  the  occurrence  or  extent  of  paralysis.  It  is  more 
common,  however,  after  the  faucial  than  after  any  other  form. 
The  paralysis  is  stated  to  occur  in  about  8  to  10  per  cent, 
of  all  cases  of  diphtheria. 

The  early  adoption  of  the  serum  treatment  of  diphtheria 
may  materially  diminish  the  liability  to  paralysis,  especially 
of  a  severe  type.  Rolleston 2  states  that  the  onset  of  post- 
diphtheritic paralysis  varies  from  4*9  per  cent,  when  antitoxin 

1  Williamson,  Lancet,  1905.  -  Rolleston,  Practitioner,  1909. 

10 


L46  NERVOUS  DISEASES 

is  given  on  the  first  day,  to  31-4  per  cent,  when  its  adminis- 
tration is  delayed  until  the  fifth  day. 

Symptoms.  By  far  the  most  frequent,  and  usually  the 
earliest,  symptom  is  paralysis  of  the  soft  palate,  which 
gives  rise  to  a  nasal  tone  of  voice  and  a  tendency  for 
fluids  to  regurgitate  through  the  nose.  When  tested,  it  will 
be  found  that  patients  are  unable  to  blow  out  the  cheeks,  or 
to  suck  or  gargle.  On  examining  the  throat,  the  palate  may 
hang  lower  than  usual,  it  may  or  may  not  be  insensitive,  but 
the  palatal  reflex  is  always  diminished  or  lost. 

In  slight  cases  the  paralysis  may  be  confined  to  this  region, 
and  gradual  recovery  takes  place  within  two  or  three  weeks. 
In  severer  cases  paralysis  of  the  glottis,  characterised  by  loss 
of  voice  and  inability  to  cough,  with  impairment  of  sensibility 
is  found.  In  other  cases  paralysis  of  the  pharynx  permits 
food  to  pass  into  the  glottis. 

Cycloplegia,  or  paralysis  of  accommodation  with  preserva- 
tion of  the  light-reflex  of  the  iris,  does  not  occur  before  the 
fourth  or  fifth  week.  Other  cranial  nerves  are  rarely 
paralysed,  except  the  sixth ;  and  ill-sustained  conjugate 
movements  of  the  globes  may  also  be  observed. 

Paralysis  of  the  limbs  may  coincide  with  the  palatal  and 
ocular  paralyses ;  but  usually  appears  about  the  fifth  or  sixth 
week.  In  some  cases,  especially  nasal  diphtheria,  the  palate 
may  escape,  but  the  extremities  suffer  earliest. 

The  legs  are  first  affected,  and  later  on  the  arms,  inter- 
costal, and  trunk  muscles.  When  atrophy  occurs  it  is  usually 
rapid,  and  the  muscles  show  degenerative  electrical  changes. 
Palsy  of  the  diaphragm  and  of  the  heart  muscle  are  the  most 
serious  symptoms,  and  in  severe  cases  come  on  early  in  the 
course  of  the  disease. 

The  sensory  symptoms  are  slight,  and  consist  of  numb- 
ness and  paresthesia ;  but  muscular  lryperassthesia  is  neither 
common  nor  well  marked.  Objective  sensory  disturbances 
of  the  peripheral  type  may  be  found. 

The  deep  reflexes  are  diminished  or  lost.  The  knee  jerk 
is  in  abeyance  from  the  commencement,  and  may  remain 
absent  from  three  to  eight  months,  after  all  other  signs  of 
the  disease  have  disappeared. 

The  sphincters  are  rarely  affected. 

The    prognosis    is    good,    provided    that    the    respiratory 


THE  PERIPHERAL  NERVOUS  SYSTEM  147 

muscles  are  not  affected,  and  that  the  heart's  action 
remains  normal.  The  palatal  paralysis  resolves  in  from  two 
to  three  weeks,  and  the  palsy  of  the  limbs  in  about  three  or 
four  months,  though  the  knee  jerks  may  remain  in  abeyance 
for  eight  months  or  longer. 

The  treatment  is  that  of  multiple  neuritis.  Complete  rest 
in  bed  is  essential,  ample  nourishment  is  necessary,  and 
stimulants  may  be  required.  Care  should  be  taken  to  prevent 
food  entering  the  larynx  :  if  there  is  severe  pharyngeal 
paralysis,  it  may  be  necessary  to  feed  through  the  nose. 
Hypodermic  injections  of  strychnine  are  valuable. 

There  is  no  satisfactory  evidence  to  show  that  injections 
of  antitoxin  have  any  remedial  effect  upon  the  symptoms  of 
paralysis  once  they  have  arisen. 

ACUTE  TOXIC  POLYNEURITIS 

Cases  resembling  Landry's  paralysis,  following  influenza, 
have  been  variously  interpreted.  In  several  such  personally 
observed,  in  which  lumbar  puncture  yielded  no  evidence 
of  organismal  infection  of  the  central  nervous  system,  the 
motor  and  sensory  symptoms  pointed  to  a  multiple  neuritis. 

The  clinical  picture  presented  by  these  cases  shows,  in  the 
first  place,  a  slight  degree  of  numbness  in  the  hands  and  feet 
without  any  muscular  hyperesthesia  or  objective  sensory  loss. 
This  is  rapidly  succeeded  by  motor  weakness  in  the  arms  and 
legs  of  wide  distribution,  affecting  both  the  proximal  and 
distal  parts,  but  predominating  in  the  extensor  groups.  At 
this  stage  the  deep  reflexes  rapidly  diminish,  and  are  soon 
lost.  Within  two  or  three  days  muscular  wasting  of  slight 
degree  but  wide  extent,  with  electrical  degenerative  altera- 
tions, appears.  Synchronously  with  this,  complaint  is  made 
of  pains  and  muscular  tenderness,  and  loss  or  diminution  to 
all  forms  of  sensation  in  the  peripheral  portions  of  the 
limbs  is  detected.  The  motor  weakness  may  also  affect  the 
muscles  of  the  trunk,  thorax,  and  in  some  cases  the  face; 
and  a  case  seen  with  such  paralyses  before  the  onset  of 
the  sensory  symptoms  is  clinically  indistinguishable  from 
one  of  Landry's  paralysis.  The  sensory  changes,  however, 
become  so  definite  that  the  diagnosis  is  made  sufficiently 
clear.     Recovery   is    usually  rapid   and    complete,  the  deep 

10  * 


148  NERVOUS   DISEASES 

reflexes  remaining  in  abeyance  for  some  time  after  the 
motor  power  has  returned.  It  would  seem  that  in  these 
cases  the  toxic  action  fell  first  upon  the  motor  cells  in 
the  spinal  cord,  and  later  gave  rise  to  parenchymatous 
neuritis.     (See  Table,  p.  448.) 

Barnes  !  and  "Williamson  ~  have  described  under  the  term 
'  Toxic  Degeneration  of  the  Lower  Neurones  '  a  condition  which 
arises  after  acute  febrile  affections.  It  is  characterised  in  the 
early  stages  by  slight  sensory  symptoms,  and  is  attended  by 
great  atrophy  of  the  small  muscles  of  the  hands.  Recovery 
is  slow  as  regards  paralysis  and  atrophy,  and  in  one  case  two 
relapses  occurred. 

This  condition  would  appear  to  us  to  resemble  that  just 
described  under  acute  toxic  polyneuritis,  but  to  differ  from  it 
in  the  fact  that  the  course  of  the  malady  is  of  a  subacute 
or  chronic  character,  and  that  the  changes  fall  particularly 
upon  the  small  muscles  of  the  hands. 

In  one  case  submitted  to  post-mortem  examination,  the 
morbid  appearances  consisted  of  degeneration  of  the  peripheral 
nerves  of  the  arms  and  atrophy  of  the  anterior  horn  cells. 

Various  forms  of  local  neuritis  are  also  observed  after 
acute  febrile  attacks.  The  most  common  varieties  occur  in 
connexion  with  the  brachial,  sciatic,  and  occipital  nerves. 

Meralgia  paraesthetica,  or  pain  and  tenderness  along  the 
external  cutaneous  nerve,  is  also  found  in  this  association. 
Probably  of  the  same  character  are  the  '  tender  toes,'  so 
common  in  typhoid  fever. 

LEPROUS    NEURITIS 

This  is  a  rare  disease  in  this  country,  but  is  from  time  to 
time  seen  in  persons  who  have  resided  in  localities  in  which 
leprosy  is  endemic. 

Pathology.  In  the  early  stages  of  the  disease,  some 
enlargement  of  the  nerves  may  be  detected,  but  when  the 
malady  is  more  advanced,  or  the  neuritis  of  old  standing, 
atrophy  of  the  affected  nerve  trunks  is  the  rule.  The 
characteristic  change  in  the  nerves  is  an  interstitial  neuritis, 
due  to  the  presence  of  the  leprosy  bacillus  in  the  meshes  of 
the  peri-  and  epineurium.     The  infection  of  the  nerves  by  the 

1  Barnes,  Brain.  1902.  -  Williamson.  Brain,  1903. 


THE  PERIPHERAL  NERVOUS  SYSTEM  L49 

bacillus  is  the  outstanding  pathological  feature  of  this  form  of 
neuritis.  Small  foci  of  bacilli  are  detected  in  the  interstitial 
tissues,  more  especially  in  relation  to  the  neurilemma  cells. 
Secondary  parenchymatous  degeneration  of  the  myeline 
sheaths  is  caused  by  their  pressure  upon  the  nerve  fibres,  as 
well  as  by  the  overgrowth  of  the  connective  tissues  of  the 
nerve  trunks.  The  bacilli  are  found  in  most  organs  and 
tissues  of  the  body,  but  are  not,  as  a  rule,  detected  in  the 
central  nervous  system. 

Symptoms.  The  symptoms  of  leprous  neuritis  are  slow 
and  progressive  in  character.  In  a  case  observed  by  ourselves, 
fifteen  years  elapsed  between  the  first  sign  of  paralysis  and 
death  from  an  intercurrent  pulmonary  affection.  The  leading 
clinical  features  of  the  disease  are  progressive  atrophic 
paralysis,  anassthesia  of  the  distal  portions  of  the  limbs 
or  of  irregular  and  patchy  distribution,  and  trophic  changes 
in  the  extremities. 

These  symptoms  may  not  be  confined  to  the  limbs,  but 
may  be  referred  to  the  nerves  of  the  trunk  and  face. 

The  motor  symptoms  consist  of  a  slowly  progressive  atrophic 
paralysis  of  irregular  distribution,  though  often  affecting 
muscles  supplied  by  a  particular  nerve.  Paralysis  of  any  one 
muscle  is  slow  and  incomplete  until  an  advanced  stage  of  the 
disease.  This  is  probably  accounted  for  by  the  escape  of 
some  nerve  fibres.     The  reaction  of  degeneration  is  present. 

The  sensory  symptoms  are  subjective  and  objective.  The 
subjective  are  often  slight,  pain  is  rare,  but  numbness  and 
tingling  are  met  with  in  most  cases.  The  objective  changes 
are  variable,  not  only  as  regards  their  distribution,  but  also 
in  the  quality  of  the  sensory  loss.  In  general  terms  it  may 
be  stated  that  the  sensory  loss  is  found  over  the  distal 
portions  of  the  limbs,  but  local  patches  of  more  or  less 
complete  loss  may  be  found  in  association  with  more  advanced 
lesions.  Epicritic  loss  may  alone  be  present,  or  both  epicritic 
and  protopathic,  in  which  case  trophic  sores  are  common. 
Loss  of  deep  sensibility  may  also  be  present. 

The  state  of  the  reflexes  depends  upon  the  position  of  the 
nerve  lesions. 

The  shin  changes  consist  chiefly  of  discoloration  of  a 
bronze  appearance,  occurring  in  patches.  Localised  thicken- 
ings, or  prominences  of  the  skin  of  the  face,  ears,  and  other 


150  NERVOUS  DISEASES 

parts,  occur  from  the  local  deposition  of  bacilli  in  the 
subcutaneous  tissues.  The  nerve  trunks  may  be  felt  on 
palpation  when  hypertrophied  in  the  early  stages. 

The  diagnosis  has  to  be  made  mainly  from  spinal  gliosis 
and  syringomyelia.  The  association  of  atrophic  paralysis — 
sensory  changes  consisting  of  the  characteristic  dissociation 
of  thermal  and  painful  from  tactile  sensibility — and  trophic 
alterations  in  the  extremities,  are  common  to  both  disorders. 

The  detection  of  the  leprosy  bacillus  in  the  subcutaneous 
tissues  places  the  diagnosis  beyond  dispute. 

The  course  of  the  disease  is  towards  a  fatal  termination 
from  intercurrent  disorder,  and  treatment  is  of  little  avail, 
except  in  the  management  of  symptoms  or  complications. 

BERIBERI 

This  is  a  disease  endemic  in  some  Eastern  countries,  but 
from  time  to  time  taking  on  an  epidemic  character.  Sporadic 
cases  are  occasionally  seen  in  this  country  amongst  sailors 
from  the  East.  It  is  due  to  the  presence  of  a  micro-organism, 
which  has  been  found  in  many  of  the  organs  and  tissues 
of  infected  persons.  It  would  appear  to  effect  an  entrance 
into  the  body  mainly  through  the  alimentary  canal,  probably 
from  the  consumption  of  rice.  Its  habitat  has  been  shown 
to  be  the  duodenum,  where  it  grows  and  multiplies,  and 
whence  a  toxic  substance  is  introduced  into  the  circulation. 
The  development  of  the  disease  is  favoured  by  states  of 
impaired  nutrition,  bad  hygienic  surroundings,  and  exposure 
to  cold  and  damp.  It  affects  males  more  often  than  females, 
and  young  adults  in  preference  to  older  people. 

Symptoms.  Gastro-intestinal  symptoms — such  as  loss  of 
appetite,  a  feeling  of  epigastric  oppression,  nausea,  and 
vomiting,  accompanied  by  malaise,  proneness  to  fatigue,  and 
palpitation — usually  precede  the  onset  of  the  nervous  symp- 
toms. These  are,  in  their  main  features,  similar  to  those  which 
have  been  described  under  alcoholic  neuritis.  Numbness, 
paresthesia,  and  muscular  hyperesthesia  usher  in  the  onset  of 
motor  weakness  in  the  hands  and  feet.  The  tendon  jerks 
are  abolished,  cutaneous  sensibility  is  impaired,  and  the 
electrical  excitability  of  the  affected  muscles  is  impaired 
or  lost. 


THE  PERIPHERAL  NERVOUS  SYSTEM  151 

In  severer  cases,  rapid  atrophic  paralysis  with  the  reaction 
of  degeneration  develops,  and  the  palsy  extends  from  the 
limbs  to  the  muscles  of  the  trunk.  Complete  paralysis 
supervenes  with  great  pain  and  muscular  tenderness,  and 
death  may  occur  from  exhaustion  or  intercurrent  disease. 

A  special  feature  of  beriberi  is  the  early  involvement  of 
the  cardio-vascular  system.  The  heart's  action  is  rapid  and 
irregular,  the  arterial  tension  falls,  and  oedema  of  the 
extremities,  sometimes  with  effusion  into  the  serous  cavities, 
is  a  characteristic  feature  of  most  cases. 

Course.  The  average  duration  of  the  common  type 
varies  from  one  to  several  months.  The  disease  is  not 
usually  fatal  except  at  the  height  of  an  epidemic.  Death 
may  occur  from  exhaustion,  heart  failure,  or  intercurrent 
disease. 

Treatment  is  based  on  the  principles  laid  down  for  other 
forms  of  peripheral  neuritis.  The  tendency  towards  cardiac 
asthenia  requires  the  administration  of  stimulants  and  heart 
tonics. 

PROGRESSIVE  HYPERTROPHIC  INTERTESTIAL 
NEURITIS 

This  is  a  rare  disease,  originally  described  by  Dejerine  and 
Sottas.  It  is  characterised  pathologically  by  thickening  of 
the  nerve  roots,  enlargement  of  the  spinal  ganglia,  and  increase 
in  volume  of  the  peripheral  nerves.  The  thickening  of  the 
nerves  is  due  to  an  overgrowth  of  the  fibrous  tissue  of  the 
nerve  bundles  and  nerve  sheaths,  with  resulting  atrophy  and 
disappearance  of  the  nerve  fibres.  The  spinal  cord  may 
present  secondary  degenerative  changes,  especially  in  the 
posterior  columns.  The  muscles  also  show  changes  in  their 
structure,  consisting  of  disappearance  of  the  normal  striation, 
and  the  replacement  of  the  muscular  fibres  by  fat  and 
fibrous  tissue. 

The  clinical  phenomena  consist  of  lightning  pains,  ataxia 
with  muscular  atrophy,  marked  sensory  changes,  and  hyper- 
trophy of  those  nerve  trunks  which  can  be  felt  under  the  skin, 
Nystagmus,  reflex  pupillary  immobility  and  kypho-scoliosis  are 
also  observed. 

The   leading   clinical   features    of    the    disease,   therefore. 


152  NERVOUS  DISEASES 

include  generalised  amyotrophy,  kyphoscoliosis,  and  tangible 
hypertrophy  of  the  nerve  trunks.  The  tendon  reflexes  are 
lost,  and  the  sphincters  are  unaffected. 

The  disease  somewhat  resembles  tabes  dorsalis,  from  which, 
however,  it  may  be  distinguished,  clinically,  by  the  absence  of 
sphincter  impairment  and  the  presence  of  definite  hyper- 
trophy of  the  peripheral  nerves,  and  pathologically  by  the 
characteristic  alterations  in  the  nerve  fibres. 

Miscellaneous  forms  of  neuritis 

Oppenheim  and  others  have  described  a  form  of  multiple 
neuritis  occurring  in  old  age,  associated  with  arterio-sclerosis. 
In  these  cases  the  symptoms  of  neuritis  are  slight  and  of 
slow  onset,  and  the  prognosis  is  not  unfavourable.  Multiple 
neuritis  has  also  been  stated  to  occur  as  a  result  of  poisoning 
by  carbon  monoxide,  sulphide  of  carbon,  and  the  coal-tar 
products — such  as  sulphonal,  trional,  antipyrin,  and  the 
anilines. 

Other  forms  of  toxic  neuritis,  in  addition  to  those  already 
described,  are  found  in  connexion  with  septicemia,  the 
puerperium,  gonorrhoea,  malaria,  and  tuberculosis. 

Local  neuritis  is  not  uncommon  in  association  with 
various  conditions — such  as  rheumatism,  gout,  tubercle, 
syphilis,  and  carcinoma. 

Tobacco.  In  chronic  tobacco-poisoning,  various  symptoms 
may  be  present — vertigo,  tachycardia,  nausea,  pain  after  food, 
insomnia,  neuralgia  and  general  tremulousness.  More  cha- 
racteristic, although  less  common,  are  interference  with  the 
heart's  action  and  amblyopia  from  retro-bulbar  neuritis. 
The  symptoms  of  the  latter  are  impairment  of  vision  and 
central  scotoma  for  green,  which  in  severe  cases  may  increase 
so  as  to  impair  all  colour  sense. 


Chaftek   VI 

NEURALGIA 

Neuralgia  is  a  clinical  term  signifying  pain  in  a  nerve. 
The  term  has  been  applied  loosely  to  all  forms  of  nerve  pain, 
irrespective  of  the  presence  or  absence  of  obvious  lesion  of  the 


THE  PERIPHERAL  NERVOUS  SYSTEM  153 

nerve  elements.  While  fully  recognising  the  impossibility  of 
determining,  in  many  cases,  whether  nerve  pain  is  due  to  an 
organic  lesion,  or  not,  it  is  advisable  to  differentiate  tentatively 
between  three  types  :  — 

(a)  Neuralgia  unassociated  with  either  present  or  previous 
organic  disease. 

(b)  Neuralgia  associated  with  organic  disease — gout,  rheu- 
matism, tabes  dorsalis,  and  neuritis. 

(c)  Neuralgia  following  upon  organic  disease — such  as 
malaria,  influenza,  herpes  zoster,  and  neuritis. 

Etiology.  Many  conditions  predispose  to  neuralgia.  It 
may  come  on  at  any  age,  but  childhood  and  old  age  are  less 
prone  to  it  than  middle  life.  Women  would  appear  to  be 
more  liable  than  men.  It  is  more  common  in  persons  of 
nervous  temperament,  and  in  the  weak,  the  anremic,  and  the 
debilitated.  Gouty  and  rheumatic  subjects  are  peculiarly 
susceptible. 

The  exciting  causes  are :  the  infective  disorders — more 
especially  malaria  and  influenza;  poisons  introduced  from 
outside  the  body — such  as  lead  and  alcohol ;  poisons  formed 
within  the  body — as  in  gout  and  diabetes;  exhausting  diseases, 
and  exposure  to  cold  and  damp. 

The  influence  of  mental  and  physical  strain,  of  overwork, 
anxiety,  and  distress  is  often  a  potent  factor  in  its  causation. 

Pains  of  a  neuralgia-like  character  may  be  found  in 
association  with  organic  disease  of  the  central  and  peripheral 
nervous  system,  in  traumatic  lesions  of  the  nerves,  in  com- 
bination with  osteo-arthritis,  and  in  herpes  zoster. 

Pain  may  be  referred  to  various  regions  of  the  body — at 
some  distance  from  the  actual  seat  of  disease  in  an  organ  or 
viscus.  These  are  not  genuine  neuralgias,  but  are  known  as 
'  referred  pains.' 

Symptoms.  Neuralgia  as  a  symptom  occurs  either  as 
pain  limited  to  one  nerve  or  its  branches,  or  as  an  element  in 
general  nervous  debility.  In  most  cases  of  neuralgia,  certain 
painful  points  on  pressure  may  be  discovered.  These  are 
found  where  cutaneous  branches  penetrate  bony  canals  or 
fibrous  structures.  These  are  usually  the  seats  of  the  greatest 
pain,  although  pain  may  also  affect  the  nerve  trunk  and  its 
branches. 

The  character  of  the  pain  varies.     In  true  neuralgia  it  is 


154  NERVOUS  DISEASES 

paroxysmal.  Sometimes  it  is  dull,  boring,  or  burning.  At 
other  times  it  is  sharp  and  lancinating,  darting  through  the 
limb,  face,  or  body.  The  duration  of  the  attacks  varies  from 
a  few  seconds  to  several  minutes,  and  their  frequency  from 
two  or  three  times  daily  to  several  in  an  hour.  In  some 
forms  of  neuralgia  the  pain  may  be  very  severe,  and  accom- 
panied by  cutaneous  hyperesthesia,  so  that  the  patient  is 
unable  to  bear  the  pressure  of  the  bed-clothes  ;  and  in  the 
trigeminal  form,  the  slightest  breath  of  cold  air  may  start 
a  paroxysm  of  pain. 

The  pain  may  be  accompanied  by  a  feeling  of  numbness 
or  deadness  in  the  affected  limb. 

Vaso-motor  symptoms  are  not  uncommon.  These  may 
take  the  form  of  patches  of  erythema,  irregularly  scattered 
over  the  neuralgic  area.  Angio-neurotic  oedema  has  been  seen 
in  association  with  severe  attacks,  and  subcutaneous  lumps 
or  nodules  of  a  transient  character  have  also  been  observed. 

The  most  characteristic  feature  of  neuralgia  is  its  tendency 
to  relapse.  In  the  malarial  and  post-influenzal  varieties  the 
relapse  may  occur  at  a  definite  and  fixed  time,  and  pass  off  as 
the  clay  advances,  to  return  at  the  corresponding  hour  next 
day.  The  malarial  variety  of  neuralgia  is  most  prone  to  affect 
the  supra-orbital  and  sciatic  nerves:  post-influenzal  neuralgia 
especially  the  supra-orbital  branch  of  the  trigeminus. 

Diagnosis.  Neuralgia  requires  to  be  distinguished  mainly 
from  neuritis.  In  the  latter  condition  the  pain  is  constant 
rather  than  paroxysmal,  and  the  nerve  is  tender  usually 
throughout  the  greater  part  of  its  extent ;  in  severer  forms  of 
neuritis  paralysis,  wasting,  and  anaesthesia  are  present. 

A  form  of  pain,  not  unlike  that  which  is  characteristic  of 
neuralgia,  may  be  found  as  a  purely  psychical  condition, 
increased  by  mental  and  emotional  causes,  and  relieved  by 
suggestion  and  distraction. 

Neuralgic  pains  are  found  as  symptoms  of  many  organic 
diseases  of  the  nervous  system— such  as  tabes  dorsalis, 
tumours  pressing  upon  the  spinal  nerve  roots,  and  chronic 
meningeal  lesions.  It  is  therefore  important  to  eliminate 
such  diseases  before  the  diagnosis  of  neuralgia  is  made. 

Prognosis.  The  prognosis  is  that  of  the  different  forms 
of  neuralgia  to  be  presently  described. 

Treatment.     In  the  first  place,  attention  should  be  paid 


THE  PERIPHERAL  NERVOUS  SYSTEM  L55 

to  the  cause  of  the  disease,  and  treatment  directed  to  the 
underlying  predisposition.  All  exciting  causes  should,  as  far 
as  possible,  be  removed.  Tonics  may  be  administered,  either 
in  the  form  of  quinine,  iron,  arsenic,  and  strychnine ;  or  as 
the  glycerophosphates — Easton's  syrup,  or  cod-liver  oil.  In 
special  forms  of  neuralgia,  such  as  the  malarial  and  influenzal, 
no  remedy  surpasses  quinine,  both  as  a  prophylactic  and  as 
an  analgesic — 5  grs.  of  quinine  taken  half  an  hour  before  the 
onset  of  the  attack  of  pain  will  often  prevent  its  occurrence. 
Salicylate  of  soda,  salicine,  aspirin,  phenacetin,  antipyrin, 
and  the  other  analgesics,  may  all  be  used  from  time  to  time 
in  the  treatment  of  this  affection. 

Cases  due  to  syphilis  and  lead,  or  of  obviously  gouty 
or  rheumatic  cause,  should  be  treated  according  to  general 
principles. 

Massage,  hydropathy,  the  application  of  the  galvanic 
current  and  vibration  may  also  be  of  use  in  particular  cases, 
or  at  some  period  in  the  course  of  the  disease.  Nerve 
stretching,  neurectomy,  excision  of  the  posterior  root  ganglia, 
or  division  of  the  posterior  nerve  roots  may  be  necessary  in 
otherwise  intractable  cases. 


TRIGEMINAL  NEURALGIA  (TIC  DOULOUREUX) 

This  is  a  painful  disease  of  the  fifth  nerve,  unassociated 
with  a  definite  or  well-established  morbid  anatomy,  although 
in  most  cases,  in  which  the  Gasserian  ganglion  has  been 
subsequently  examined,  an  interstitial  fibrous  overgrowth  has 
been  found.  The  ganglion  cells  are,  as  a  rule,  of  normal 
appearance.  Thickening  of  the  nerve  sheath  and  endarteritis 
have  also  been  observed. 

The  disease  is  one  of  adult  and  late  adult  life,  beginning 
between  the  ages  of  forty  and  fifty,  and  continuing  for  many 
years.     It  occurs  about  equally  in  the  sexes. 

Symptoms.  It  is  characterised  by  paroxysms  of  intense 
pain,  lasting  a  second  or  two,  and  continued  for  a  few  hours 
or  days,  over  prolonged  periods.  The  distribution  of  the  pain 
is  over  one  or  more  of  the  branches  of  the  fifth  nerve.  It  may 
come  on  sometimes  without  any  cause :  at  other  times  on 
attempting  to  speak,  smile,  or  laugh ;  when  eating,  while  cleans- 
ing the  teeth,  or  occasionally  from  a  breath  of  cold  air  upon  the 


156  NERVOUS  DISEASES 

cheek.  The  pain  ma}7  affect  one  or  more  spots  ;  sometimes  it 
is  the  nose  or  the  eye,  or  it  may  be  the  jaw,  or  the  side  of  the 
tongue,  or  the  roof  of  the  mouth.  From  the  starting-place  it 
may  radiate  over  the  branch  mainly  affected.  The  starting- 
points  are  those  where  a  branch  of  the  nerve  becomes  sub- 
cutaneous. These  spots  are  tender  to  the  slightest  pressure 
in  some  cases,  but  in  others  no  such  tenderness  is  detected. 

The  paroxysms  of  pain  are  usually  accompanied  by 
flushing  of  the  face  on  the  affected  side  ;  there  is  often  a 
shiny  or  greasy  appearance  of  the  skin  ;  sometimes  the  cheek 
is  puffy  or  cedematous ;  there  may  be  a  profuse  secretion  of 
tears,  photophobia,  and  increase  of  the  salivary  secretion. 
Tremors  or  twitchings  of  the  facial  muscles  may  accompany 
the  paroxysms. 

The  facial  appearance  of  these  patients,  after  the  disease 
has  persisted  for  a  time,  is  often  characteristic.  There  is  an 
expression  of  stolidity  from  a  fear  of  emotional  display — as 
the  slightest  facial  movement  may  induce  a  painful  paroxysm. 
The  mouth  is  foul  and  the  breath  offensive — as  attempts  to 
cleanse  the  teeth  give  rise  to  pain.  In  well-marked  cases  the 
jaws  are  edentulous — as  the  teeth,  carious  and  healthy  alike, 
have  been  by  degrees  extracted  in  the  vain  hope  of  curing 
the  malady. 

Diagnosis.  Tne  well-established  disease  offers  no  diffi- 
culty in  diagnosis,  but  in  the  early  stages  and  in  all  cases  of 
facial  neuralgia,  the  possibility  of  carious  teeth,  of  malignant 
disease,  or  of  suppuration  in  the  sinuses  (frontal,  maxillary, 
ethmoidal)  should  be  borne  in  mind  as  not  improbable  causes 
of  the  pain. 

Prognosis  and  course.  The  disease  may  run  a  prolonged 
course  over  many  years,  with  no  tendency  to  spontaneous 
cure.  Although  the  paroxysms  may  recur  at  frequent  inter- 
vals, long  periods  of  freedom  from  pain,  amounting  to  several 
months  or  years,  are  not  uncommon.  The  tendency  in  the 
severe  cases  is  for  the  relapses  to  occur  with  increasing 
frequency. 

The  treatment  consists  of  both  medical  and  surgical 
methods.  The  only  certain  cure  of  the  disease  lies  in 
excision  of  the  Gasserian  ganglion.  This  operation  has  now 
been  proved  in  some  hundreds  of  cases  to  be  entirely  satis- 
factory, recurrence  of  the  pain  rarely,  if  ever,  taking  place. 


THE  PERIPHERAL  NERVOUS  SYSTEM  L57 

Medicinal  remedies  by  the  mouth  are  of  little  use  except 
as  palliatives.  The  most  efficacious  are  quinine,  in  two  to  five 
grain  doses,  thrice  daily;  tincture  of  gelsemium  or  aconite 
pushed  to  the  production  of  toxic  symptoms,  and  butyl- 
chloral,  ten  to  fifteen  grains,  repeated  about  every  four  hours. 
Analgesics — such  as  antipyrin,  phenacetin,  phenalgin,  exalgin, 
and  aspirin — may  be  of  temporary  use,  but  cannot  be  regarded 
as  in  any  sense  trustworthy  remedial  agents.  Colchicum  has 
given  relief  in  gouty  cases  of  a  plethoric  nature. 

Injections  of  substances  into  the  nerve  have  been  resorted 
to  lately,  with  considerable  benefit  in  many  of  the  recorded 
cases.  This  method  has  been  advocated  as  being  less 
dangerous  than  excision  of  the  Gasserian  ganglion,  while 
affording  a  more  permanent  relief  than  can  be  obtained 
by  drugs.  Two  solutions  have  been  used  successfully : 
(1)  5-10  minims  of  80-per-cent.  alcohol;  (2)  5  minims  of 
a  2-per-cent.  solution  of  osmic  acid.  These  may  be  injected 
into  the  branches  of  the  nerve  as  they  issue  from  their 
respective  foramina. 

Subcutaneous  injections  of  morphia  and  cocaine  are  not 
to  be  recommended,  and  should  only  be  given  under  critical 
and  exceptional  circumstances. 

The  local  application  of  galvanism  (positive  pole  over  the 
tender  points)  is  sometimes  attended  by  highly  satisfactory 
results,  but  in  other  instances  the  pain  may  be  increased. 

SCIATIC  NEURALGIA— SCIATICA 

Pain  in  the  distribution  of  the  great  sciatic  nerve  and  its 
branches  is  a  common  ailment,  more  especially  in  men  of 
adult  and  middle  age.  In  contrast  to  brachial  neuralgia,  it 
is  more  frequent  upon  the  left  side. 

Some  cases  would  appear  to  be  instances  of  neuralgia,  but 
the  majority  are  due  to  an  inflammatory  affection  of  the  sheath 
of  the  nerve — a  perineuritis.  In  all  old-standing  or  inveterate 
cases,  especially  if  accompanied  by  much  lameness,  deformity, 
or  wasting  of  the  limb,  a  careful  examination  of  the  hip 
joint  should  be  made,  as  sciatic  pain  is  a  common  accompani- 
ment of  osteo- arthritis  of  the  hip  joint,  just  as  brachial 
neuralgia  is  frequently  associated  with  a  similar  affection  of 
the  shoulder  joint. 


158 


NERVOUS  DISEASES 


Etiology.  The  disease  is  often  hereditary,  and  is 
predisposed  to  by  gout  and  rheumatism.  The  great  exciting 
cause  is  exposure  to  cold  and  damp,  especially  in  those 
undergoing  prolonged  physical  strain.  Bilateral  sciatic  pain 
is  suggestive  of  disease  within  the  spinal  canal,  of  malignant 
or  tuberculous  disease  of  the  sacrum  and  pelvis,  and  of  osteo- 
arthritis of  the  hip  joints. 

Symptoms.  Pain  of  a  constant  gnawing  character,  in 
the  position  of  the  trunk  of  the  nerve  or  its  branches,  with 
tenderness  on  pressure  along  the 
nerve  or  in  certain  places,  is  the 
chief  characteristic  of  sciatica.  The 
tender  points  are :  (a)  over  the  sacro- 
sciatic  notch  ;  (b)  behind  the  great 
trochanter ;  (c)  in  the  middle  of 
the  thigh  posteriorly ;  (d)  behind 
the  head  of  the  fibula,  and  on  the 
peroneal  aspect  of  the  leg  ;  and  (<•) 
on  the  outer  side  of  the  ankle  or 
foot. 

At  the  onset  of  an  attack,  the 
pain  may  be  so  severe  as  to  confine 
the  patient  to  bed;  but  in  the  later 
stages  it  becomes  intermittent,  being 
readily  induced  by  fatigue,  exercise, 
or  any  sudden  strain  or  effort  of 
the  leg. 

The  pain  is  increased  by  move- 
ments of  the  limb,  by  bending  the 
body  at  the  hips,  or  by  flexing  the  hip  with  the  leg  extended 
at  the  knee,  and  in  walking.  Hence  a  characteristic  attitude 
of  the  leg  is  assumed,  the  hip  and  knee  joint  being  slightly 
flexed,  and  the  heel  raised  from  the  ground.  Owing  to  the 
sense  of  comfort  brought  about  in  this  way,  contracture  of 
the  leg — sometimes  of  a  very  troublesome  character — is 
induced. 

The  pain  induced  by  pressure  on  the  nerve  in  sitting 
favours  a  posture  in  which  the  patient  rests  upon  the  tuber 
isehii  of  the  sound  side. 

As  a  rule  muscular  atrophy  is  absent,  although  in  old- 
standing   cases   a  general  wasting    of    the  whole  limb  may 


Fi 


.  53. — Photograph  of  a 
case  of  old-standing 
sciatica  of  the  right  leg, 
showing  slight  general 
muscular  wasting:. 


THE  PERIPHERAL  NERVOUS  SYSTEM  1 59 

be  observed.  Unless  some  degree  of  neuritis  is  present,  no 
sensory  changes  are  detected.  "When  present  they  consist  of 
impairment  of  the  epicritic  sensibility  over  the  sole  and 
outer  side  of  the  dorsum  of  the  foot,  and  the  peroneal 
aspect  of  the  leg.  The  knee  jerks  are  brisk.  In  the  neuralgic 
cases  the  Achilles  jerks  are  normal ;  in  the  neuritic  cases,  on 
the  other  hand,  the  Achilles  jerk  on  the  affected  side  is 
abolished,  and  may  remain  in  abeyance  even  long  after  the 
pain  has  disappeared. 

Obvious  muscular  wasting,  with  impairment  of  and  quali- 
tative changes  in  the  electrical  irritability  of  the  muscles  and 
decided  sensory  loss,  point  to  a  sciatic  neuritis. 

Diagnosis.  Pain  along  the  sciatic  nerve  may  be  due  to 
several  conditions  other  than  sciatic  neuralgia.    These  are  :— 

1.  Sciatic  neuritis. 

2.  Disease  of  the  hip  joint. 

3.  Lesions  of  the  cauda  equina. 

4.  Malignant  disease  of  the  pelvic  organs. 

Sciatic  neuritis  is  characterised  by  muscular  wasting, 
sensory  impairment,  abolition  of  the  ankle  jerk,  nerve 
tenderness,  and  altered  electrical  excitability  of  the  muscles. 

In  disease  of  the  hip  joint  the  use  of  the  Rontgen  rays  is 
most  valuable  in  revealing  changes  in  the  head  of  the  femur 
or  the  acetabulum.  The  two  affections  of  this  joint  most 
commonly  observed  are  tuberculous  disease  and  osteo-arthritis. 
In  both  the  movements  of  the  joint  are  restricted,  and  the 
limb  may  be  slightly  shortened  and  rotated  inwards.  Pain 
on  pressure  along  the  nerve  is  rare.  Rheumatoid  changes  in 
other  joints  may  also  be  observed. 

In  lesions  of  the  cauda  equina,  whether  arising  from 
primary  intraneural  disease  or  from  caries  of  the  sacrum, 
muscular  wasting  with  electrical  alterations,  segmental 
sensory  disturbances  and  sphincter  weakness,  are  usually 
sufficiently  obvious  to  make  the  diagnosis  clear  (p.  120). 

The  prognosis  of  the  malady  varies,  as  sciatic  pain  may 
persist  over  many  months.  One  of  its  features,  as  in  all 
cases  of  neuralgia,  is  a  tendency  to  relapse,  apparently 
without  any  discoverable  reason.  We  have  seen  sudden 
turning  in  bed  bring  on  a  relapse  more  severe  than  the 
original  affection.  Post-herpetic  sciatica  is  a  severe  variety 
of  the  malady,  but  pursues  a  more  continuous  course  towards 


160  NERVOUS  DISEASES 

recovery.  A  cure  ma}'  be  guaranteed  in  all  cases  of  true 
sciatica. 

Treatment.  Sciatica  is  a  very  troublesome  malady,  and 
often  taxes  tbe  resources  of  the  physician.  In  the  early 
stages  rest  in  bed  is  essential,  when  hot  applications  and 
mild  counter-irritants  may  be  applied  to  the  leg.  Radiant 
heat  applications  and  '  mud  baths '  are  also  of  benefit,  when 
administered  daily  for  a  time.  Internally,  iodide  of  potassium, 
salicylate  of  soda,  salicine,  and  aspirin  may  be  prescribed 
with  benefit.     Injections  of  morphia  may  be  necessary. 

After  the  acute  stage  has  passed  away,  massage,  passive 
movements  of  the  limb,  especially  if  contracture  is  present, 
and  sometimes  galvanism,  are  of  great  service.  The  thermo- 
cautery or  blistering,  applied  over  the  tender  point  or  along 
the  line  of  the  nerve,  has  often  been  of  use  in  relieving  pain 
and  discomfort. 

Sulphur  appears  to  have  in  many  cases  almost  a  specific 
action,  and  may  be  prescribed  either  internally,  as  sulphur 
water,  or  in  the  form  of  sulphur  baths. 

Injections  of  chloroform,  osmic  acid,  and  alcohol  into  the 
nerve  have  been  advocated. 

In  old-standing  or  inveterate  cases,  following  upon  an 
inflammation  of  the  nerve  sheath,  the  nerve  may  be  exposed 
and  the  sheath  freed,  or  nerve-stretching  may  be  attempted 
after  its  exposure  by  surgical  methods.  The  majority  of 
cases  resolve  without  any  surgical  assistance. 

OTHER  FORMS  OF  NEURALGIA 

Cervico-occipital  neuralgia  is  a  condition  characterised 
by  pain  at  the  back  of  the  head  and  neck,  and  extending 
upwards  over  the  scalp  towards  the  vertex.  It  is  accompanied 
by  tenderness  along  the  great  occipital  nerve  and  the  posterior 
branches  of  the  cervical  plexus.  The  scalp  is  often  peculiarly 
tender  and  sensitive  to  brushing  the  hair,  and  mental  depres- 
sion is  a  not  uncommon  accompaniment.  The  pain  is  usually 
bilateral,  and  often  of  a  constant  dull  aching  type  with 
paroxysmal  exacerbations.  Pain  is  increased  on  movement  of 
the  head. 

Brachial  neuralgia  (brachialgia)  is  a  common  form  of 
neuralgia.     Complaint  is  made  of  intense  pain,  chiefly  in  the 


THE  PERIPHERAL  NERVOUS  SYSTEM  161 

shoulder,  but  also  extending  down  the  arm  to  the  tips  of  the 
fingers,  usually  of  the  fore  and  middle  fingers.  It  is  always 
increased  by  movements  of  the  arm.  Most  of  the  nerves  are 
tender  on  pressure.  In  less  acute  cases  the  pain  may  be 
limited  to  the  distribution  of  the  circumflex  nerve,  to  the 
bend  of  the  elbow,  or  to  the  nerves  about  the  wrist  and 
palm  of  the  hand. 

The  presence  of  a  brachial  neuralgia,  whether  affecting  the 
whole  limb  or  only  the  hand  and  wrist,  may  lead  to  inability 
to  write.  The  existence  of  this  disability  gives  rise  to  the 
impression  that  the  patient  has  writer's  or  other  form  of 
professional  neurosis.  It  is  more  frequently  seen  in  the 
right  arm. 

In  all  cases  of  persistent  brachial  neuralgia  the  shoulder 
joint  should  be  carefully  examined,  as  osteo-arthritic  changes 
are  not  uncommonly  found  in  association  with  it. 

Intercostal  and  abdominal  neuralgia.  If  pain  is  in  the 
left  mammary  region,  the  patient  is  troubled  with  the  idea 
of  heart  disease ;  while  in  the  abdomen  it  may  lead  to  the 
impression  that  appendicitis  or  organic  visceral  disease  is 
the  cause  of  the  pain.  It  is  increased  by  the  respiratory 
movements.  If  the  upper  dorsal  nerves  of  the  left  side  are 
affected,  pain  resembling  that  of  angina  pectoris  may  be  felt 
along  the  ulnar  border  of  the  arm  and  hand. 

It  is  usually  accompanied  by  marked  tenderness  on  pressure 
along  the  intercostal  spaces  ;  but  especially  to  one  side  of  the 
vertebral  spines,  in  the  mid-axillary  region,  and  near  the 
costo-sternal  articulations. 

It  is  a  common  antecedent  and  sequel  of  herpes  zoster. 
The  lower  abdominal  nerves    (ilio-inguinal  and  ilio-hypo- 
gastric)  are  also  the  seat  of  occasional  neuralgia.     The  usual 
tender  points  are  present.     It  may  extend  as  a  dull,  aching, 
boring  pain  into  the  testicular  region  and  groin. 

Anterior  crural  neuralgia.  Pain  in  this  nerve  may  occur 
alone,  or  as  part  of  a  generalised  neuralgic  affection  of  the 
whole  limb.  It  would  seem  to  be  especially  prone  to  occur  in 
men  from  the  fatigues  of  long  marches,  mountain  climbing,  or 
other  muscular  overaction  of  the  lower  limbs.  It  is  sometimes 
an  early  symptom  of  disease  of  the  bones  of  the  dorso-lumbar 
region  of  the  spine.  The  nerve  is  tender  to  pressure  within 
Scarpa's  triangle,  and  standing  and  walking  are  impaired. 

11 


162  NERVOUS  DISEASES 

Mastodynia  is  the  term  given  to  a  painful  condition  of 
the  breast  in  women.  It  occurs  during  pregnancy,  lac- 
tation, and  in  association  with  disease  of  the  female  pelvic 
organs.  It  may  lead  to  an  obsession  or  fear  of  cancer  of  the 
breast. 

The  pain  may  be  severe,  and  is  usually  constant  with 
paroxysmal  exacerbations.  The  breast  may  be  red  and  swollen, 
and  the  nipple  sensitive  and  tender.  It  is  relieved  by  the 
removal  of  the  associated  cause. 

Coccygodynia.  Neuralgia  of  the  coccygeal  nerves  is  rare. 
Pain  in  the  coccyx  is,  however,  common.  It  is  a  local 
symptom  of  a  general  disorder — such  as  hysteria  or  neuras- 
thenia— and  is  frequently  due  to  a  fall  upon  or  injury  to  the 
lower  part  of  the  spine. 

There  is  marked  tenderness  on  pressure  over  the  coccyx, 
and  pain  is  also  complained  of  in  other  parts  of  the  spine, 
which  may  be  tender.  The  treatment  is  that  for  hysteria. 
Although  the  coccyx  has  been  excised,  pain  may  continue 
after  its  removal. 


Chaptee  VII 

HERPES   ZOSTER 

Herpes  zoster  is  an  inflammatory,  often  hemorrhagic, 
condition  affecting  the  posterior  root  ganglia,  accompanied  by 
pain,  sensory  disturbances,  and  the  presence  of  a  characteristic 
vesicular  eruption  in  the  area  of  distribution  of  the  affected 
nerve  or  nerves.  It  may  be  either  a  primary  condition,  or 
symptomatic  of  interference  with  the  functions  of  the  posterior 
ganglia  from  carious  or  malignant  disease  of  the  bones  of 
the  spine. 

Pathology.  The  observations  of  Head  and  Campbell l 
have  shown  that  the  changes  in  this  condition  consist  of  an 
exudation  of  small  round  cells  and  extravasation  of  blood 
within  the  ganglia  upon  the  posterior  roots.  In  the  centre 
of  the  hemorrhage  or  inflammatory  focus,  the  ganglion  cells 
and  fibres  are  completely  destroyed,  while  at  the  periphery  the 

1  Head  and  Campbell,  Brain,  1900. 


THE  PERIPHERAL  NERVOUS  SYSTEM  L63 

cells  are  swollen,  structureless,  and  stain  abnormally.  Inflam- 
matory changes  are  also  seen  in  the  sheath  of  the  ganglion. 

In  slight  cases  these  changes  may  clear  up  without  leaving 
any  obvious  scar ;  but  in  the  severe  forms,  regressive  changes 
are  observed  in  the  extravasated  blood,  and  the  affected  focus 
becomes  replaced  by  fibrous  scar  tissue. 

The  changes  found  in  the  posterior  nerve  roots  correspond 
to  the  severity  of  the  ganglionic  lesion,  and  consist  of  an 
acute  degeneration  followed  by  a  greater  or  less  amount  of 
sclerosis. 

In  the  peripheral  nerves  close  to  the  ganglia,  acute  de- 
generation of  the  fibres  is  seen,  with  breaking  up  of  the 
myeline  sheaths,  especially  in  the  posterior  primary  divisions  of 
the  nerves.  In  the  severe  cases  marked  sclerosis  of  the  nerve 
may  result.  In  a  few  cases  hemorrhage  and  inflammation 
may  actually  occur  in  the  peripheral  nerve.  The  acute 
degenerative  products  are  found  to  be  rapidly  absorbed  and 
the  secondary  sclerosis  quickly  ensues.  No  changes  are  found 
in  the  anterior  nerve  roots  and  motor  fibres. 

In  the  spinal  cord,  degenerated  fibres  corresponding  to 
the  affected  ganglia,  may  be  found  in  the  posterior  root 
zone. 

Similar  changes  are  found  in  the  secondary  and  sympto- 
matic zosters,  arising  from  implication  of  the  posterior  root 
ganglia  in  malignant,  tuberculous,  and  traumatic  affections  of 
the  spine.  Herpes  is  also  stated  to  occur  in  association  with 
tabes  and  paretic  dementia  (Head). 

The  shin  changes  are  also  of  an  inflammatory  character 
and  consist  of  an  eruption  of  vesicles,  which  usually  contain 
a  clear  serous  fluid,  and  whose  floor  is  formed  by  inflamed 
papillse.  Degenerated  nerves  have  also  been  found  passing 
towards  the  areas  of  affected  skin. 

An  inflammation  of  the  lymphatic  glands  is  also  seen  in 
these  cases,  the  axillary  glands  being  affected  when  the  lesion 
is  above  the  seventh  dorsal  nerve  and  the  inguinal  when  it 
is  below  this  level. 

Symptoms.  The  malady  usually  arises  without  any 
obvious  peripheral  or  central  cause.  The  patient  feels  ill, 
suffers  considerable  malaise  and  pain,  and  the  temperature 
is  elevated.  These  symptoms  may  last  for  three  or  four 
days,  after  which,  in  most  cases,  the  characteristic  vesicular 

11  * 


164 


NERVOUS  DISEASES 


eruption  appears.     In  many  cases,  however,  the  eruption  is 
preceded  for  some  days  by  severe  local  pain  and  sometimes 
hyperesthesia  over  the  distribution  of  the  affected  nerve. 
The  commonest  situations  are  the  cervical  region,  particu- 
larly the  third  and  fourth 
cervical  nerves,  and   from 
the    third    dorsal    to    the 
second      lumbar     nerves, 
especially     the     third, 
fourth,    and    fifth    dorsal. 
The  trigeminal  and  facial 
nerves      (Gasserian      and 
geniculate     ganglia)     may 
also  be  affected. 

The  eruption  is  distinc- 
tive in  character.  It  con- 
sists of  groups  of  vesicles, 
arising  from  an  erythema- 
tous base,  occurring  in 
crops  and  containing  fluid. 
This  may  be  clear,  or  in 
severe  cases  bloodstained  ; 
and  in  others  it  may 
be  purulent.  It  appears 
along  the  distribution  of 
the  nerve  root,  although 
not  necessarily  affecting 
the  whole  root  area.  It 
spreads  with  varying 
rapidity,  but  appears  first 
and  is  most  severe  over 
the  posterior  primary,  the 
lateral  and  anterior 
branches  of  the  anterior 
may  break  down  and  form 
the  tenth  day  after  their 
to  dry  up  and  form  scabs, 
may  be    left,    but    in    the 


Fig. 


54. — Photograph    of 
herpes  zoster. 


a    case    of 


primary  divisions.     The  vesicles 
an    ulcer.      From    the   fifth   to 
appearance,  the  vesicles    begin 
In   the    milder    cases    no    trace 


more  severe  forms  white  scars  take  the  place  of  the  scabs 
and  persist-     (Fig.  54.) 

During  the  period  of  the  eruption  the  pain  may  be  of  a  severe 


THE  PERIPHERAL  NERVOUS  SYSTEM  165 

and  burning  character,  and  worse  at  night.  In  a  few  cases, 
especially  in  old  people,  it  may  persist  for  an  indefinite 
time. 

"While  the  rash  is  present  painful  sensibility  and  the 
severer  degrees  of  heat  and  cold  are  abolished  in  a  small 
portion  of  the  affected  area ;  but  in  the  peripheral  parts 
hyperalgesia  may  be  found.  Tactile  sensibility  is  unaffected, 
and  the  whole  area  may  even  be  hypersesthetic.  After 
the  disappearance  of  the  rash  there  is  little  or  no  interference 
with  sensation,  except  where  scarring  is  present,  when  some 
general  impairment  of  all  forms  of  sensation  may  be  found. 

A  second  attack  rarely  occurs,  and  a  bilateral  distribution 
of  the  eruption  is  rare. 

The  second,  third,  and  fourth  cervical  nerve  root  areas 
are  frequently  affected  together  ;  and  occasionally  two  or  more 
contiguous  roots  in  other  parts  of  the  body,  although,  as  a 
rule,  the  eruption  is  confined  to  one  root  area. 

The  diagnosis  does  not  present  any  difficulty. 

The  prognosis  as  regards  recovery  from  the  attack,  is 
good.  In  a  few  cases,  especially  the  senile  forms,  neuralgic 
pain  of  a  severe  character  may  persist. 

The  treatment  consists  chiefly  in  the  application  locally 
to  the  eruption  of  soothing  and  sedative  ointments  and 
powders — such  as  oxide  of  zinc,  boracic  ointment  with  cocaine 
and  starch  powder.  The  eruption  may  be  advantageously 
covered  with  collodion  to  protect  it  from  external  influences. 
Internally  antipyrin,  phenacetin,  or  aspirin  may  be  given 
for  the  relief  of  pain,  which  if  severe,  however,  requires 
morphia. 

The  application  of  galvanism  along  the  nerve  may  be  found 
of  use  in  some  cases  of  persistent  post-herpetic  pain. 


Part  VI 
DISEASES   OF   THE   BRAIN 

Chapter  I 
THE   INTRACRANIAL   BLOOD    SUPPLY1 

The  brain  is  supplied  by  two  arteries  :  the  basilar,  formed 
by  the  union  of  the  vertebral  arteries,  and  the  internal  carotid 
artery.  The  basilar  artery  gives  origin  to  the  posterior 
cerebral  artery,  which  sends  off  a  small  posterior  com- 
municating branch  to  the  internal  carotid.  The  internal 
carotid  artery  divides  into  the  middle  and  anterior  cerebral 
arteries.  The  two  anterior  cerebral  arteries  are  joined 
together  by  the  anterior  communicating  artery,  in  this  way 
completing  the  circle  of  Willis.  All  these  blood-vessels  in 
their  course  give  off  numerous  smaller  arterioles,  which  pass 
into  the  brain  and  supply  its  several  parts. 

Two  points  in  connexion  with  the  vascular  supply  require 
mention.  First,  no  anastomoses  exist  between  the  arteries 
supplying  respectively  the  cortex  and  the  deeper  structures. 
There  is  therefore  a  hard-and-fast  line  of  demarcation  between 
the  superficial  and  the  deep  areas.  Secondly,  the  three 
cortical  systems  intercommunicate  with  each  other  at  the 
confines  of  their  respective  areas ;  but  the  individual  branches 
of  the  cortical  arteries  are  end-arteries,  and  do  not  anastomose 
with  their  contiguous  branches. 

The  simplest  method  of  description  is  to  take  each  primary 
artery  and  refer  to  its  distribution  :  first  upon  the  cortex  of 
the  brain,  and  secondly,  in  the  subcortical  structures.  The 
written  account  should  be  read  and  studied  in  conjunction 
with  the  figures.     (Figs.  55-58.) 

1  The  account  given  here  is  based  upon  the  paper  of  Dr.  Beevor. — Brain,  1907. 

166 


DISEASES  OF  THE  BRAIN  167 

I.  Anterior  cerebral  artery 

(a)  Superficial  distribution.  This  embraces  the  mesial 
surface  from  the  frontal  pole  as  far  as,  or  about  one  inch 
short  of,  the  internal  parietooccipital  fissure,  including  the 
whole  of  the  marginal  gyrus,  the  gyrus  fornicatus,  the  corpus 
callosum,  and  the  orbital  surface  of  the  frontal  lobe.  On  the 
external  surface  it  extends  as  far  back  as  the  middle  of 
the  parietal  lobe,  and  as  far  downwards  as  the  level  of  the 
superior  frontal  sulcus. 


Fig.  55. — The  vascular  supply  of  the  convexity  of  the  brain.  A 
(horizontal  lines) ,  anterior  cerebral  artery.  B  (vertical  lines) .  middle 
cerebral  artery.  C  (oblique  lines),  posterior  cerebral  artery. 
P.O.,  external  parietal-occipital  fissure.      r,  fissure  of  Rolando. 

(b)  Deep  distribution.  This  includes  the  inferior  half  of 
the  head  of  the  caudate  nucleus  and  anterior  limb  of  the 
internal  capsule,  the  anteroinferior  part  of  the  external  and 
sometimes  the  second  segment  of  the  lenticular  nucleus,  the 
floor  of  the  third  ventricle,  and  portions  of  the  anterior 
commissure  and  anterior  pillar  of  the  fornix. 

This  artery  also  supplies  the  mesial  half  of  the  centrum 
ovale  of  the  frontal  lobe,  as  far  back  as  the  caudate  nucleus. 

2.  Middle  cerebral  artery 

(a)  Superficial  distribution.  The  upper  limit  of  the 
middle  cerebral  supply  upon  the  convexity  is  bounded  in 
front  by  the  superior  frontal  sulcus,  and  behind  by  the 
intraparietal  fissure  and  anterior  occipital  sulcus. 


168 


NERVOUS  DISEASES 


Its  posterior  limit  varies  with  that  of  the  anterior  border 
of  the  posterior  cerebral  supply,  but  in  most  cases  it  reaches 
as  far  as,  or  just  short  of,  the  occipital  pole.  Its  lower  limit 
lies  along  the  middle  of  the  inferior  temporal  convolution,  or 
the  second  temporal  sulcus.  It  also  supplies  the  outer  half  of 
the  orbital  surface  of  the  frontal  lobe. 

{b)  The  dee})  distribution  includes  the  middle  and  external 


Fig.  56. — Vascular  supply  of  the  mesial  surface  of  the  hemisphere. 
The  lettering  is  the  same  as  in  fig.  55.  D  (clotted  area),  anterior 
choroidal  artery. 

segments  of  the  lenticular  nucleus,  the  posterior  limb  of  the 
internal  capsule,  the  remainder  of  the  caudate  nucleus,  and 
the  whole  of  the  centrum  ovale  corresponding  to  the  extent  of 
the  superficial,  or  cortical  supply.  This  comprises  the  outer 
third  of  the  frontal  segment,  the  external  capsule  and  insula, 
the  Eolandic  and  parietal  segments  and  the  outer  and  upper 
half  of  the  occipital  segment  of  the  central  white  matter,  and 
the  optic  radiations. 

3.  Posterior  cerebral  artery 

(a)  The  superficial  distribution  extends  over  the  whole  of 
the  ventro-mesial  surface  of  the  temporo-occipital  lobe  as  far 
as  the  internal  parieto-occipital  fissure,  or  even  to  a  short 
distance  in  front  of  it.  It  does  not,  however,  supply  the 
uncinate  convolution,  which  receives  a  branch  from  the 
anterior  choroidal  artery.  It  extends  round  the  occipital  pole 
for  a  distance  of  a  half-inch  on  to  the  external  surface,  but 


DISEASES  OF  THE  BRAIN  109 

superiorly  may  extend  as  far  forwards  as  the  external  parieto- 
occipital fissure  or  even  farther,  and  abut  against  the  anterior 
cerebral  distribution. 

(b)  Deep  distribution.  Posterior  two-thirds  of  the  pes 
pedunculi,  the  red  nucleus,  the  posterior  half  of  the  optic 
thalamus,  the  pulvinar,  and  the  lower  and  inner  segment  of 
the  optic  radiations. 

A 


Fig.  57. — Vascular  supply  of  the  interior  of  the  hemisphere,  at 
the  level  of  the  anterior  limb  of  the  internal  capsule.  The 
letterine1  is  the  same  as  in  fig's.  55  and  56. 


4.  The  anterior  choroidal  artery 

(a)  Superficial  distribution.  This  is  limited  to  the 
uncinate  lobule. 

(b)  Deep  distribution.  The  anterior  part  of  the  optic 
thalamus,  the  greater  portion  of  the  fornix,  the  corpora - 
quadrigemina  and  the  internal  and  external  geniculate  bodies  ; 
the  posterior  two- thirds  of  the  posterior  limb  of  the  internal 
capsule,  the  internal  segment  of  the  lenticular  nucleus,  the 
retrolenticular  fibres  of  the  internal  capsule  and  the  origin 
of  the  optic  radiations. 

5.  The  cerebellar  arteries 

The  cerebellum  is  supplied  by  three  arteries  derived  from 
the  vertebral  and  basilar  arteries.     The  inferior  cerebellar 


170  NERVOUS  DISEASES 

branch  of  the  vertebral  artery  winds  round  the  medulla 
oblongata,  and  supplies  the  under  surface  of  the  cerebellum. 
The  middle  cerebellar  artery  is  given  off  from  the  basilar, 
and  is  distributed  to  the  under  surface  of  the  cerebellum. 
The  superior  cerebellar  artery — a  branch  of  the  basilar — 
winds  round  the  crus  cerebri,  and  is  distributed  over  the 
superior  surface  of  the  cerebellum. 


Fig.  58. — The  vascular  supply  of  the  interior  of  the  hemisphere 
at  the  level  of  the  optic  thalamus  and  posterior  limb  of  the 
internal  capsule.  The  lettering  is  the  same  as  in  the 
preceding   figure. 


6.  The  bulbar  arteries 

The  pons  and  medulla  are  richly  supplied  with  vessels 
derived  from  the  basilar  artery,  which  penetrate  its  substance 
both  mesially  and  laterally.  Larger  branches  of  the  ver- 
tebral and  basilar  arteries  also  are  conveyed  into  the  bulb 
along  the  roots  of  the  cranial  nerves.  These  arteries  are  all 
end-arteries,  and  find  their  way  through  the  bulb  to  the 
grey  matter  of  the  floor  of  the  fourth  ventricle. 

7.  The  venous  return 

The  venous  blood  from  the  brain  returns  by  a  number 
of  sinuses,  situated  mainly  in  the  dura  mater,  to  the  internal 
jugular  vein.     A  large  number  of  veins  from  the  convexity 


DISEASES  OF  THE  BRAIN  171 

enter  the  superior  longitudinal  sinus,  which  passes  backwards 
to  the  torcular  Herophili.  The  blood  from  the  interior  of 
the  brain,  the  ventricles,  and  callosal  region  returns  by  the 
inferior  longitudinal  sinus,  the  veins  of  Galen,  and  the 
straight  sinus  to  the  torcular.  The  superior  longitudinal  and 
straight  sinuses  fuse  in  this  locality  with  the  large  lateral 
sinus,  which  is  derived  from  the  cavernous  and  inferior 
petrosal  sinuses,  into  which  the  cerebellar  veins  also  open. 

It  should  be  remembered  that  the  intracranial  sinuses  are 
also  in  direct  communication  with  veins  from  the  face  and 
scalp,  from  the  nose  and  orbit,  from  the  mastoid  bone,  and 
from  the  diploe  of  the  cranial  bones. 


Chaptee  II 
GENERAL    SYMPTOMS    OF    BRAIN   DISEASE 

Before  entering  upon  a  description  of  the  diseases  of  the 
brain,  it  is  necessary  to  refer  briefly  to  the  symptoms  of 
cerebral  disease  in  general,  as  these  depend  more  upon  the 
seat  than  upon  the  nature  of  the  lesion. 

The  symptoms  of  cerebral  disease  are  shown  by  an 
interference  with  the  functions  of  the  brain  in  three  ways : 
(a)  by  excess  of  function  (motor  convulsion  and  sensory 
paresthesia)  ;  (b)  by  diminution  or  abolition  of  function 
(motor  and  sensory  paralysis) ;  and  (c)  by  a  combination  of 
impairment  and  excess  of  function. 

In  order  to  comprehend  the  factors  underlying  the  pro- 
duction of  the  symptoms  of  brain  disease,  it  should  be  borne 
in  mind  that  the  cerebral  cortex  is  the  highest  centre  of  the 
nervous  system,  that  in  it  are  received  and  perceived  the 
several  forms  of  sensibility,  general  and  special,  that  it  is 
the  centre  for  voluntary  movement,  and  that  it  controls  to 
some  extent  the  lower  centres.  Its  integrity  is  essential  for 
the  development  and  evolution  of  all  mental  processes.  It 
is,  therefore,  obvious  that  all  kinds  of  brain  disorder  may, 
irrespective  of  the  local  disturbance  due  to  their  situation, 
lead  to  interference  with  the  functions  of  the  brain  as 
a  whole. 


172  NERVOUS  DISEASES 

The  highest  centres  of  the  brain  occupy  a  relatively  large' 
area  in  the  grey  matter  of  the  cerebral  cortex,  and  are 
localised  to  certain  well  recognised  regions.  Experimental, 
histological,  pathological,  and  clinical  investigations  have 
permitted  the  mapping  out  on  the  surface  of  the  brain,  the 
position  of  the  centres  for  motion,  sensation,  vision,  hearing, 
taste,  and  smell,  as  well  as  more  specialised  centres  for  speech 
and  writing.  Some  of  these  centres  are  in  juxtaposition,  and 
others  are  some  distance  apart. 

Cortical  lesions.  As  the  centres  of  voluntary  motion  and 
of  general  and  special  sensation  are  situated  in  the  cerebral 
cortex,  cortical  lesions  will  cause  disturbance  of  function, 
characterised  by  symptoms  of  an  irritative  character,  so  long 
as  the  cortical  elements  are  not  destroyed.  Cortical  lesions 
are,  therefore,  characterised  by  symptoms  of  irritation — focal 
epilepsy — either  motor  or  sensory,  according  to  the  situation 
of  the  lesion  ;  and  are  accompanied  or  followed  by  paralytic 
symptoms  of  a  temporary  or  permanent  character  (hemiplegia, 
hemianesthesia,  hemianopsia,  &c). 

Subcortical  lesions.  In  passing  from  the  cerebral  cortex  to 
the  brain  stem  and  spinal  cord,  the  tracts  conveying  im- 
pressions to  and  from  the  cortex  are  in  closer  anatomical 
association.  In  the  internal  capsule,  the  motor,  sensory,  and 
visual  fibres  are  arranged  in  this  order  from  before  back- 
wards (pp.  3,  4).  A  lesion  in  this  locality  may,  therefore, 
give  rise  to  motor,  sensory,  and  visual  symptoms  of  a  para- 
lytic character,  as  nerve  tracts,  and  not  nerve  centres, 
are  involved.  The  paralytic  symptoms  will,  however, 
be  wide  and  extensive,  although  the  loss  of  power  in 
any  one  part  is  not  so  great  as  in  complete  destruction  of 
its  cortical  centre. 

The  motor  and  sensory  tracts  pass  through  the  crura 
cerebri  in  their  passage  to  and  from  the  internal  capsule 
and  pons.  Symptoms  of  lesion  of  the  crus,  therefore, 
may  be  hemiplegic  paralysis  and  hemianesthesia  of  the 
contralateral  side,  with  homolateral  paralysis  of  the  third 
cranial  nerve. 

Unilateral  lesions  of  the  pons,  if  high  up,  may  cause 
hemiplegia  with  or  without  hemianesthesia.  If  the  lesion  be 
situated  below  the  decussation  of  the  fibres  to  the  facial  nucleus, 
the  face  may  not  be  paralysed. 


DISEASES  OF  THE  BRAIN  173 

HEMIPLEGIA 

In  hemiplegia  paralysis  is  present  on  the  side  of  the  body 
contralateral  to  the  cerebral  lesion.  The  paralysis  is  one  of 
movement  and  not  of  muscles.  The  actions  of  contralateral 
muscles,  which  are  only  used  in  association  with  correspond- 
ing homolateral  muscles,  are  less  impaired  than  the  actions 
of  contralateral  muscles  acting  solely  on  the  contralateral 
side.  This  explains  the  relatively  slight  impairment  of  the 
intercostal  and  masseter  muscles  on  the  contralateral    side. 

This  is  probably  due  to  the  bilateral  representation  of 
bilateral  movements  in  the  cerebral  cortex  and  to  a  connexion 
between  the  bulbar  and  spinal  centres  of  the  two  sides. 

From  this  it  might  be  supposed  that  bilateral  movements 
would  be  impaired  on  both  sides  of  the  body,  and  such  is 
found  to  be  the  case. 

A  further  point  of  interest  is  that  certain  movements,  such 
as  respiration,  are  normally  automatic  and  innervated  chiefly 
from  lower  centres  in  the  bulb.  In  addition,  voluntary 
respiratory  movements  may  occur — as  in  taking  a  deep 
inspiration ;  such  movement  being  innervated  from  the  cere- 
bral cortex.  As  Hughlings  Jackson  pointed  out,  in  a  patient 
suffering  from  recent  hemiplegia,  during  ordinary  respiration 
the  paralysed  side  of  the  chest  expands  more  than  the  non- 
paralysed  :  whereas  if  the  patient  be  asked  to  take  a  deep 
breath,  the  non-paralysed  side  expands  more  than  the 
paralysed. 

Paralysis  of  the  action  of  certain  muscles  may  occur  upon 
the  same  side  as  the  lesion  (homolateral),  when  such  action 
is  used  to  effect  a  lateral  movement  towards  the  contralateral 
hemiplegic  side.     Notable  examples  of  this  are  :  — 

1.  The  impairment  of  rotation  of  the  head  to  the  contra- 
lateral side.  This  movement  is  chiefly  performed  by  the 
sterno-mastoid  muscle  on  the  homolateral  side  acting  along 
with  other  less  important  muscles  on  the  contralateral  side. 
That  the  depressor  action  of  the  homolateral  sterno-mastoid 
muscle  is  not  paralysed  may  be  demonstrated  by  making  the 
patient  depress  his  head  against  resistance.  It  will  be  found 
that  the  head  deviates  towards  the  contralateral  side,  owing 
to  the  weakness  of  the  contralateral  sterno-mastoid  muscle. 

2.  The  impairment  of  conjugate  lateral  movement  of  the 


171  NERVOUS  DISEASES 

eyes  to  the  contralateral  side.  This  movement  is  effected  by 
the  external  rectus  muscle  of  the  contralateral  side  acting  in 
conjunction  with  the  internal  rectus  muacle  of  the  homo- 
lateral side.  In  hemiplegia  there  is  no  paralysis  of  the 
convergent  action  of  either  internal  rectus  muscle. 

In  a  typical  case  of  hemiplegia,  the  arm,  leg,  and  the 
lower  part  of  the  face  are  paralysed.  The  tongue,  when 
protruded,  deviates  to  the  paralysed  side,  and  the  voluntary 
movements  of  the  bilateral  trunk  muscles  are  also  impaired. 
Thus,  when  the  patient  tries  to  sit  up,  the  umbilicus  is  drawn 
towards  the  non-paralysed  side. 

The  paralysis  of  the  face  may  be  complete  for  voluntary, 
emotional,  or  associated  movements.  In  cases  where  the 
lesion  is  in  the  neighbourhood  of  the  optic  thalamus,  emotional 
movements  only  may  be  affected,  whereas  lesions  in  other 
situations  may  show  paralysis  of  voluntary  and  associated 
movements  with  little  or  no  impairment  of  emotional 
movement. 

The  loss  of  power  is  usually  greater  in  the  arm  than  in 
the  leg,  and  greater  in  the  extremity  of  the  limb  than 
towards  the  trunk.  As  a  general  rule  the  leg  recovers 
before  the  arm.  Cases  in  which  the  leg  is  more  severely 
paralysed  are  due  to  lesions  of  the  motor  fibres  to  the  leg  ;  the 
commonest  site  of  such  lesion  is  the  posterior  part  of  the 
internal  capsule.  As  the  sensory  and  visual  fibres  are 
in  close  relation  to  the  fibres  for  the  leg  in  this  region,  hemi- 
anesthesia and  hemianopsia  are  common  accompaniments 
(p.  4,  fig.  4). 

Associated  movement  sometimes  occurs  in  the  paralysed 
limb.  If  a  patient  with  hemiplegia — who  is  unable  to  move, 
for  example,  the  left  hand— be  made  to  close  the  right  hand 
tightly  some  movement  of  the  paralysed  left  hand  may  be 
observed.  Much  less  frequent,  but  more  striking,  is  the  fact 
that  occasionally  a  patient — one  of  whose  arms  is  completely 
paralysed  for  voluntary  movement  —  may,  when  yawning, 
raise  his  paralysed  hand  to  his  mouth. 

Rigidity.  Gowers  has  described  three  types  of  rigidity : 
(a)  Initial  rigidity,  which  may  be  present  at  the  onset  of 
hemiplegia  and  only  lasts  for  an  hour  or  two.  (b)  Early 
rigidity,  which  commences  within  a  few  days  of  the  onset, 
and   lasts   for   one   to   four    or    five  weeks  :  this  rigidity  is 


DISEASES  OF  THE   BRAIN 


175 


M 


Fig.  59. 


Fig.  60. 


Fig.  61. 

Figs.  59-61  show  the  face  at  rest,  smiling,  and  laughing,  in 
a  case  of  right-sided  hemiplegia ;  the  lesion  being  in  the 
neighbourhood  of  the  optic  thalamus. 


176  NERVOUS  DISEASES 

slight  in  degree  and  variable,  (c)  Late  rigidity  or  spasticity, 
which  comes  on  several  weeks  after  the  stroke.  Although 
it  is  present  in  all  the  muscles,  the  limbs  tend  to  become 
fixed  in  characteristic  positions  owing  to  the  greater  power 
of  certain  groups  of  muscles.  Thus  the  arm  is  adducted 
at  the  shoulder  and  flexed  at  the  elbow  ;  the  forearm  is  in 
pronation,  the  ringers  are  clenched,  and  the  thumb  flexed 
and  opposed  into  the  palm.  The  thigh  is  adducted  at  the 
hip,  the  knee  is  rigid,  the  heel  slightly  drawn  up,  the  foot 
inverted  and  the  toes  pointed. 

Later  on,  structural  changes  in  the  muscles  and  joints 
may  give  rise  to  fixation  of  the  limbs.  This  is  distinct  from 
spasticity,  and  cannot  be  overcome  by  passive  movement 
even  under  an  anaesthetic. 

Muscular  tone.  During  the  acute  stage  of  hemiplegia, 
before  the  onset  of  early  rigidity,  there  may  be  a  stage  of 
temporary  flaccidity.  Except  during  this  stage,  the  muscular 
tone  is  increased  and  with  it  the  corresponding  tendon  reflexes. 

Vaso-motor  and  trophic  changes,  For  some  time  after 
a  stroke  the  temperature  of  the  paralysed  side  is  raised  from 
one  to  two  degrees  above  the  nonparalysed.  The  paralysed 
side  may  not  present  any  vaso-motor  changes  :  on  the  other 
hand  it  may  be  red.  swollen  and  perspire  freely  ;  or  it  may 
be  cold  and  blue— the  latter  appearances  being  seen  in  the 
late  stages. 

Trophic  changes  may  be  absent,  or  may  be  slight  and 
consist  of  a  general  wasting  with  dryness  of  the  skin  :  on 
the  other  hand,  in  such  cases  as  infantile  hemiplegia  and 
extensive  cortical  lesions,  marked  atrophy  of  the  limbs  may 
be  observed. 

Disorders  of  movement.  These  may  be  convulsive, 
tonic  or  clonic,  regular  or  irregular,  or  consist  of  slow  mobile 
spasm,  depending  on  the  seat  and  nature  of  the  lesion.  The 
different  varieties  are  described  on  pp.  216  and  221. 

Sensory  symptoms.  As  with  motor  paralysis  sensory 
disturbances  may  be  hemiplegic,  monoplegic,  or  even  more 
limited.  All  forms  of  sensation  may  be  affected.  In  cortical 
lesions  the  sensory  loss  may  exist  without  motor  palsy  and 
be  of  limited  distribution.  In  lesions  involving  the  sensory 
tracts  in  the  lower  portions  of  the  brain,  motor  and  visual 
paralyses  may  coexist. 


DISEASES  OF  THE  BRAIN  177 

Reflexes.  On  the  paralysed  side  the  deep  reflexes  are 
exaggerated,  the  superficial  abdominal  reflexes  are  impaired 
or  abolished,  and  the  plantar  response  is  extensor  in 
type. 

General  symptoms,  such  as  headache,  vertigo,  and  mental 
impairment  are  frequent,  and  are  referred  to  later. 

Cranial  nerve  paralyses,  as  symptoms  of  brain  disease, 
have  been  dealt  with  in  Part  IV. 


FOCAL  EPILEPSY 

This  term  has  been  applied  to  cases  in  which  seizures  of  an 
epileptic  character  result  from  the  direct,  immediate,  or  late 
effect  of  a  lesion  of  the  brain,  the  membranes,  or  the  skull. 
This  application  of  the  term  includes  all  cases  in  which  fits 
occur  in  consequence  of  a  cerebral  lesion.  In  the  majority  of 
cases  the  attacks  are  of  local  character  and  limited  extent — 
Jacksonian  epilepsy  ;  in  ,the  remainder,  epileptic  fits  in  every 
way  resembling  those  of  the  idiopathic  disease  are  set  up  :  but 
it  is  a  common  clinical  experience  to  find  either  a  combination 
of  the  two  forms  or  a  transition  type  of  seizure.  Cases  show- 
ing either  of  these  varieties  require  careful  study — attention 
being  given  to  the  original  character  of  the  seizures  and  to  the 
physical  signs.  Focal  fits  happening  late  in  the  history  of 
a  case  may  be  false  localising  signs,  while  generalised  epilepsy 
occurring  early  may,  on  the  other  hand,  obscure  the  local 
nature  of  a  lesion. 

Jacksonian  epilepsy  may  occur  in  consequence  of  any 
organic  lesion  of  the  brain,  or  its  membranes.  If  the  lesion 
is  situated  so  as  to  press  upon  or  involve  the  cortex,  fits  are 
almost  invariable,  and  are  one  of  the  earliest  signs.  Should 
the  lesion  be  seated  deeply  convulsions  are  usually  late 
in  onset,  and,  although  often  Jacksonian  in  type,  are  more 
frequently  generalised. 

It  is  worthy  of  notice  that  in  cases  of  cerebral  syphilis,  or 
multiple  lesions  of  the  brain,  fits  with  local  commencement 
are  common ;  but  there  is  no  constancy  in  the  seat  of  com- 
mencement, the  '  discharge '  sometimes  taking  place  from  one 
cortical  area  and  sometimes  from  another. 

It  may   be  mentioned  that   in  cases  with  increase  of  the 

12 


178  NERVOUS  DISEASES 

general  intracranial  pressure  — such  as  hydrocephalus  and 
cerebral  tumour — although  generalised  epileptic  seizures  are 
the  rule,  yet  attacks  with  local  commencement  may  supervene. 
Cortical  lesions.  The  fits  in  focal  or  Jacksonian  epilepsy 
have  certain  definite  and  constant  features. 

1.  They  always  commence  in  the  same  manner  in  the  same 
part  of  the  body :  the  initial  symptoms  of  each  attack  are 
therefore  always  the  same. 

2.  The  part  of  the  body  affected  corresponds  to  the  cortical 
area  from  which  the  '  discharge  '  issues. 

3.  The  march  of  the  fit  is  in  a  more  or  less  definite  order, 
in  consequence  of  the  overflow  of  the  '  discharge  '  from  the 
primaiy  focus  to  adjacent  cortical  centres. 

4.  Consciousness  is  not  lost  except  when  the  fit  becomes 
general,  or  arises  from  lesion  in  the  highest  psychical  centres. 

The  nature  of  the  fit  varies  according  to  the  situation  of 
the  irritative  lesion.  If  in  the  frontal  region,  the  initial 
symptoms  are  psychical ;  if  in  the  motor,  convulsive  ;  if  in 
the  sensory,  paresthetic  ;  if  in  one  of  the  specialised  centres, 
visual,  auditory,  olfactory  or  gustatory,  respectively. 

1.  Epilepsy  arising  from  lesion  of  the  motor  area.  The 
cortical  discharge  results  in  convulsive  movements  starting 
in  a  particular  area  of  the  body.  These  movements  are 
brought  about  by  clonic  contractions  of  the  executive  muscle 
or  group  of  muscles.  The  contractions  commence  slowly, 
but  become  more  rapid,  tending  towards  tonic  spasm  and 
passing  off  gradually  by  a  succession  of  wider  but  less 
frequent  clonic  jerks. 

If  the  discharge  is  severe  the  clonic  may  pass  into  a  tonic 
stage,  which  passes  off  with  slow  clonic  jerkings  occurring  at 
less  frequent  intervals. 

After  the  fit  a  condition  of  exhaustion  paralysis  results. 
This  is  seen  in  weakness  of  certain  movements,  more  especially 
those  which  were  involved  most  completely  in  the  irritative 
stage. 

The  exhaustion  phenomena  are  usually  transitory;  but  a 
progressive  and  persistent  paresis  may  eventually  ensue  as  a 
result  of  permanent  injury  to  the  discharging  cortical  area. 

Focal  epilepsy,  followed  by  progressive  weakness  of  the 
parts  affected  by  the  discharge,  is  therefore  characteristic  of  a 
progressive  cortical  lesion — at  first  small  and  irritative,  later 


DISEASES  OF  THE  BRAIN  179 

large  and  destructive.  In  certain  cases,  a  sudden  onset  of 
paralysis  occurs  in  the  '  fit  area,'  due  in  most  instances  to 
the  occurrence  of  hemorrhage  into  the  primary  focus  of 
irritation. 

Jacksonian  epilepsy  may  occur  in  cases  in  which  paralysis 
already  exists,  and  it  is  striking  that  in  such  cases  the  fits 
usually  commence  by  convulsive  movements  in  the  paralysed 
muscles.  A  cerebral  lesion  may  directly  induce  the  fit,  or 
may  by  reducing  the  stability  of  the  cortical  centres  render 
them  more  liable  to  discharge  ;  and  the  greater  the  number  of 
fits,  the  less  stable  is  the  condition  of  the  cerebral  cortex. 

2.  Epilepsy  arising  from  lesion  of  the  sensory  area. 
Usually  the  fit  is  ushered  in  by  a  feeling  of  numbness — 'pins- 
and-needles,'  or  '  drawing '  of  the  skin — in  a  particular  area  ; 
more  rarely  by  a  subjective  sensation  of  movement  in  some 
part  of  the  body. 

These  sensations  spread  and  are  usually,  though  not  in- 
variably, accompanied  or  followed  by  a  motor  seizure  in  the 
corresponding  areas.  After  the  fit,  a  loss  or  diminution  of 
common  sensation  of  a  transient  character  is  detected  over 
the  affected  area.  Permanent  sensory  loss  is  brought  about 
in  a  manner  similar  to  motor  paralysis. 

3.  Epilepsy  arising  from  lesion  of  the  special  sense  areas. 
Irritative  seizures  are  rarely  highly  specialised.  In  occipital 
lesions,  subjective  visual  sensations  are  observed  in  the  corre- 
sponding visual  fields,  and  occasionally  these  are  followed  by 
transient  blurring  or  loss  of  sight.  As  a  rule  no  permanent 
impairment  of  function  results  from  the  fits,  although  such 
may  result  directly  from  the  lesion. 

Subjective  sensations  of  smell,  taste,  and  hearing  are  usually 
bilateral  and  are  not  followed  by  complete  loss  of  function. 

4.  Epilepsy  arising  from  lesion  of  the  psychical  centres. 
In  these  cases  the  highest  consciousness  is  impaired  or 
abolished.  The  patient  'loses  himself,'  as  in  'petit  mal' 
attacks.     In  other  cases  automatism  is  present. 

In  most  cases  of  Jacksonian  epilepsy,  there  is  a  tendency 
for  the  fit  to  extend  to  other  regions,  so  that  one  side  of  the 
body  may  be  involved  in  the  convulsion.  When  the  cortical 
discharge  takes  place  rapidly,  consciousness  is  often  lost,  and 
the  fit  tends  to  become  generalised,  implicating  both  sides  of 
the  body. 

12  * 


180  NERVOUS  DISEASES 

APHASIA,  OR  LOSS  OF  SPEECH 

Language,  using  the  term  in  its  widest  sense,  may  be 
taken  to  be  the  means  by  which  communication  and  exchange 
of  ideas  takes  place  between  individuals.  It  includes  gestures, 
signs,  expression,  speech,  and  writing.  Articulate  speech  is 
the  form  of  language  characteristic  solely  of  man,  and  is  the 
result  of  the  higher  evolution  of  the  human  brain. 

Before  attempting  to  describe  the  various  disorders  of 
speech,  occurring  as  the  result  of  disease,  a  general  account 
of  the  processes  of  acquisition  and  evolution  of  speech  in  the 
child  is  desirable. 

It  may  be  mentioned,  by  way  of  preface,  that  the  specialised 
centres  subserving  speech  are  developed,  in  the  majority  of 
persons,  in  the  left  cerebral  hemisphere.  This  special  function 
of  the  left  side  of  the  brain  appears  to  have  been  acquired 
along  with  right-handedness  in  the  process  of  evolution. 

The  child  at  first  commences  to  recognise  different  objects 
by  their  size,  shape,  colour,  and  touch,  and  learns  to  dis- 
tinguish them  by  the  differences  which  they  exhibit.  Later 
on,  he  associates  various  attributes  with  each  object.  In 
course  of  time  he  hears  names  applied  to  the  objects,  and 
from  their  constant  repetition  and  from  the  observation  of 
what  is  associated  with  their  use,  he  begins  to  understand  the 
meaning  of  the  words  before  he  is  able  to  intelligently 
reproduce  their  sound.  It  may  be  that  at  times  the  child 
accidentally  reproduces  a  correct  word,  and  it  is  ultimately,  by 
the  perception  of  the  correctness  of  the  sound  and  by  many 
attempts  at  its  repetition,  that  at  last  he  not  only  recognises, 
but  is  able  to  reproduce  the  word.  A  similar  process  attends 
the  acquisition  of  writing,  where  signs  are  recognised  and 
intelligently  reproduced. 

It  is  obvious  that  two  fundamental  factors  are  essential 
for  the  production  of  speech :  first,  perception  upon  the 
sensory  side,  and  secondly,  expression  upon  the  motor  side. 
The  fundamental  sensory  functions  most  intimately  con- 
nected with  speech  are  audition  and  vision ;  but,  as  will  be 
seen  from  a  further  study  of  this  subject,  the  accurate 
expression  of  the  motor  side  of  speech  depends— at  least  in  its 
earlier  stages — upon  the  perception  of  the  muscular  articula- 
tory  movements  entailed  in  their  performance.     Thus,  a  child 


DISEASES  OF  THE  BRAIN  181 

is  unable  voluntarily  to  reproduce  a  word  until  it  can  corre- 
late satisfactorily  the  sense  of  movement  produced  by  the 
correct  articulation  with  the  appreciation  of  its  sound.  It  is 
by  the  use  of  the  sense  of  movement  of  the  lips  and  tongue, 
in  articulation  in  association  with  visual  impressions,  that  the 
congenitally  deaf,  or  deaf-mutes,  learn  to  speak. 

The  motor,  or  expressive  side  of  speech,  consists  of  the 
primary  centres  in  the  precentral  gyrus  for  the  lips,  tongue, 
vocal  cords,  their  connexions  with  the  bulb,  the  bulbar  nuclei, 
the  motor  nerves,  and  the  muscles  subserving  articulation. 

Specialised  or  higher  motor  centres,  of  later  development, 
in  which  movements  associated  with  speech  and  writing  are 
solely  represented,  are  situated  apart  from  the  motor  centres 
in  the  intermediate  or  psychomotor  region  of  the  frontal  lobe. 

The  position  of  these  centres  is  probably  in  the  posterior 
ends  of  the  second  and  third  frontal  gyri — the  intermediate 
precentral  area  described  and  figured  by  Campbell. 

The  centre,  lying  within  the  third  frontal  gyrus,  is  known 
as  Broca's  area,  and  is  more  especially  associated  with  spoken 
language ;  while  the  centre  in  the  second  frontal  gyrus 
subserves  the  expression  of  written  language — the  writing 
centre  (fig.  2,  p.  3). 

The  sensory  or  receptive  side  of  speech  consists  of  two 
parts — the  auditory  and  the  visual. 

1.  The  auditory  centre.  Sound  is  conveyed  from  the  organ 
of  Corti  by  the  auditory  nerve  and  central  auditory  tract 
partly  to  the  temporal  lobe  of  the  opposite  side,  and  in  part 
to  that  of  the  same  side,  both  ears  being  bilaterally  repre- 
sented in  the  cortex.  The  auditory  centre  is  situated  in  the 
posterior  portion  of  the  first  temporal  gyrus  and  in  the  gyri 
of  Heschl.  Of  this  area,  a  certain  portion — the  audito- 
psychical  or  the  auditory  word-centre — is  located  in  the 
posterior  part  of  the  first  temporal  gyrus  (fig.  11,  p.  64). 

2.  The  visual  centre.  The  primary  visual  centres  are 
situated  in  the  cortex  of  the  occipital  lobe  around  the  calcarine 
fissure.  A  higher  visual  or  visuo-psychical  centre  is  described 
by  Campbell  as  situated  upon  the  external  surface  of  the 
occipital  lobe.  A  cortical  centre  for  the  recognition  of  written 
language  or  visual  word-centre  is  situated  in  the  angular,  and 
partly  in  the  supra-marginal  gyri. 

The  centre  for  the  sense  of  muscular  movement  of  the 


182  NERVOUS  DISEASES 

lips,  tongue,  and  vocal  organs  is  situated  in  the  post-central 
gyrus,  as  elsewhere  described  (p.  18). 

It  is  a  well-recognised  fact  that  the  centres  subserving  the 
functions  of  speech  lie  on  either  side  of  the  Sylvian  fissure. 
Around  its  posterior  end  and  corresponding  to  the  posterior 
segments  of  the  fissure,  both  above  and  below,  are  the  visual 
and  auditory  receptive  centres ;  while  anteriorly  forming 
partly  the  upper  border  of  the  fissure  and  partly  its  base  are 
Broca's  area  and  the  island  of  Eeil.  Situated  within  the 
fissure  and  supplying  the  gyri  on  both  sides  is  the  Sylvian 
artery  :  thrombotic  lesions  of  which  are  not  uncommon— a 
fact  which  explains  the  frequent  association  of  paralysis  of 
the  receptive  centres. 

Defects  of  speech  arising  from  cortical  or  subcortical 
lesions  ma}^  be  subdivided  into  the  sensory  and  motor 
aphasias.  These  two  forms  of  aphasia  may  occur  separately 
or  in  association.  Interference  with  the  sensory  side  or 
perception  of  spoken  or  written  language,  materially  impairs 
the  motor  or  expressive  side,  and  with  it  there  is  usually 
observed  some  degree  of  mental  impairment.  In  his  doctrine 
of  aphasia  recently  propounded,  Marie1  has  stated  that  lesion 
of  the  sensory  speech-centres  and  their  subjacent  white  matter 
(the  zone  of  Wernicke)  is  the  lesion  essential  to  aphasia, 
and  if  loss  of  motor  speech  is  also  present,  it  is  accounted  for 
by  a  subcortical  lesion  giving  rise  to  anarthria,  or  paralysis  of 
articulation. 

It  is,  however,  necessary  to  refer  to  the  symptoms  which 
result  from  lesion  of  one  or  other  of  the  cortical  centres  of 
speech  and  their  subcortical  connexions. 

Word  deafness.  Destruction  of  the  auditory  word- centre 
is  followed  by  word  deafness,  or  inability  to  understand 
spoken  language. 

Although  motor  speech  is  not  impossible,  the  effect  of 
destruction  of  the  auditory  word-centre  is  to  produce  a  jargon, 
which  is  not,  however,  recognised  as  such  by  the  patient. 

In  minor  degrees  of  interference  with  the  centre,  there 
exists  an  inability  to  recall  words,  or  a  tendency  to  use  the 
wrong  words  (paraphasia).  There  is  also  a  loss  of  power  of 
naming  objects,  although  the  use  or  purpose  of  the  object 
is   known,   and  can  be    expressed  in    a  roundabout  fashion. 

1  Marie,  La  semaiiie  medicale,  1906. 


DISEASES  OF  THE  BRAIN  183 

Persons  so  affected  may  be  able  to  read  aloud,  owing  to  a 
direct  connexion  between  the  visual  word-centre  and  Broca's 
area. 

The  inability  to  name  objects,  already  mentioned,  may  be 
the  first  sign  of  interference  with  the  functions  of  the 
auditory  word-centre  from  lesion  of  the  temporal  lobe.  It  has 
been  found  to  be  the  earliest  symptom  of  abscess  of  this  lobe, 
secondary  to  suppurative  middle  ear  disease,  and  it  was  also 
observed  in  a  case  of  subdural  abscess,  in  which  no  lesion  was 
present  within  the  temporal  lobe. 

The  auditory  word-centre  has  numerous  communications 
with  the  other  speech  centres,  of  which  the  following  may  be 
mentioned  with  the  symptoms  resulting  from  their  destruction. 

1.  With  the  higher  visual  centre  in  the  angular  gyrus— 
the  audito-visual  commissure.  Destruction  of  this  tract  is 
followed  by  an  inability  to  pick  out  words  or  figures  from 
print  or  writing  when  asked  to  do  so ;  although  a  person 
affected  in  this  way  would  be  able  to  read  aloud. 

2.  With  the  lower  visual  centres  in  the  occipital  lobe,  by 
the  inferior  longitudinal  fasciculus :  inability  to  pick  out 
objects  named. 

3.  With  the  motor  centres  in  the  precentral  gyrus  ; 
inability  to  repeat  spoken  words  or  phrases. 

4.  With  the  higher  motor  centre  of  the  intermediate 
precentral  or  Broca's  area :  inability  to  reply  to  spoken 
language — in  other  words,  conversation  would  be  impossible. 

5.  With  the  higher  motor  centre  of  the  intermediate 
precentral  zone  or  graphic  area :  inability  to  write  from 
dictation. 

Word  blindness.  Destruction  of  the  visual  word-centre 
results  in  word  blindness,  or  an  inability  to  understand 
written  or  printed  language.  This  has  received  the  name  of 
Alexia.  There  is  usually  also  some  interference  with  writing 
— wrong  words  being  used  or  written  in  a  wrong  way  (para- 
graphia). Although  the  power  of  recognising  letters  may  be 
lost,  the  recognition  of  figures  may  be  retained.  Inability  to 
read  aloud,  to  write  from  dictation,  or  to  copy  or  transfer 
print  into  writing  are  also  present. 

The  visual  word-centre  is  commissurally  connected  with 
other  centres  concerned  in  speech.  The  following  are  the 
main  connexions  with  their  physiological  uses. 


184  NERVOUS  DISEASES 

1.  With  the  auditory  word-centre — visno-auditory  com- 
missure. This  commissure  is  of  use  for  the  purposes  of 
reading  aloud,  except  in  strong  'visuals'  in  whom,  as  already 
mentioned,  a  direct  connexion  exists  between  the  higher 
visual  and  the  higher  speech-centre  in  Broca's  area. 

2.  "With  the  precentral  and  intermediate  precentral  centres 
for  writing :  the  former  permits  of  copying,  and  the  latter 
of  transcribing  printed  into  written  language. 

Subcortical  aphasia.  This  is  the  term  applied  to  the 
effects  consequent  upon  a  lesion  not  of  the  cortical  centres,  as 
above  described,  but  of  their  subcortical  connexions  and 
association  tracts.  An  extensive  lesion  of  this  character  brings 
about  the  most  complete  form  of  sensory  aphasia. 

If  the  lesion  is  subcortical  to  the  auditory  centre,  the 
symptoms  are:  inability  to  understand  spoken  language,  to 
name  objects,  to  pick  out  objects  or  words  named,  or  even  to 
speak,  with  a  possible  exception  in  the  case  of  a  strong  'visual.' 
If  the  lesion  is  subcortical  to  the  visual  word-centre,  the 
patient  cannot  understand  written  or  printed  language,  and 
he  can  neither  read  aloud,  write  nor  copy.  Although  he  could 
probably  name  objects,  he  would  be  unable  to  write  the  name 
or  to  recognise  it  when  written.  This  lesion  is  associated 
with  homonymous  hemianopsia  to  the  opposite  side. 

Motor  aphasia.  Motor  aphasia  consists  of  inability  to 
express  language  in  speech  or  writing,  with  retention  of 
the  power  of  understanding  what  is  said  or  written.  The 
executive  centres  for  speech  are  situated  in  the  intermediate 
precentral  zone  :  that  for  articulatory  speech  in  Broca's  area, 
and  that  for  writing  in  the  posterior  end  of  the  second  frontal 
gyrus.  Destruction  of  the  former  may  result  in  a  loss  of 
power  of  movement  for  articulatory  speech  without  coexistent 
motor  paralysis  of  the  muscles  used  in  speech  ;  of  the  latter  in 
a  loss  of  the  power  of  writing  spontaneously. 

In  this  connexion  reference  may  again  be  made  to  the 
recent  observations  of  Marie,  who  has  advanced  the  view  that 
an  isolated  lesion  of  Broca's  convolution  may  be  unassociated 
with  motor  aphasia,  and  that  when  this  symptom  exists,  the 
lesion  is  more  extensive,  and  extends  backwards  towards  the 
sensory  perceptive  centres. 

As  clinical  evidence,  however,  of  the  existence  of  these 
centres  it  may  be  pointed  out  that,  in  certain  cases  of  tumour 


DISEASES  OF  THE  BRAIN  185 

of  the  left  frontal  lobe,  fits  have  been  observed  in  which  a 
complete  temporary  loss  of  speech  without  loss  of  consciousness 
occurred.  Written  and  spoken  commands  were  understood, 
and  there  was  no  paralysis  of  voluntary  movements  of  the  face, 
tongue,  or  palate.  During  the  seizure,  the  patient  was  able 
to  signal  for  assistance  and,  later,  to  record  accurately  the 
sensations  experienced  during  the  attack.  A  further  example, 
pointing  to  the  existence  of  a  specialised  speech-centre,  was 
that  of  a  child,  who  for  about  six  months  had  been  able  to 
speak.  After  a  generalised  epileptic  fit,  permanent  loss  of 
motor  speech  ensued  without  anarthria,  motor  palsy,  or  sensory 
aphasia.  That  this  was  not  due  to  lesion  of  the  lower 
motor  centres  was  proved  by  the  ability  to  move  the  lips 
and  tongue,  and  by  imitation  to  produce  words. 

Commonly,  the  lesion  giving  rise  to  aphasia  involves  not 
only  Broca's  convolution,  but  its  connexions  with  the  motor 
centres  in  the  precentral  gyrus  and  its  subcortical  fibres.  In 
these  cases  aphasia  with  motor  paralysis  is  the  result.  In 
subcortical  lesions  of  the  left  frontal  lobe  and  adjacent  parts 
an  inability  to  speak  is  accompanied  by  a  right-sided  hemi- 
plegia. In  these  cases  there  is  no  true  aphasia,  but  anarthria 
or  paralysis  of  articulation. 

The  following  is  the  method  of  examination  of  a  case  of 
aphasia  according  to  Bastian's  scheme  : — 

1.  Ascertain  whether  the  patient  is  right-  or  left-handed. 

2.  What  is  the  state  of  his  education  as  regards  reading 
and  writing  ? 

3.  Ascertain  the  condition  of  hearing  and  of  vision. 

4.  Does  he  understand  language — whether  spoken,  written, 
or  printed  ? 

5.  Has  the  patient  spontaneous  speech — articulatory,  or 
graphic  ?  If  so,  what  is  its  nature ?  Does  he  use  wrong 
words  ? 

6.  Can  he  repeat  words  spoken  to  him  ? 
Can  he  read  what  he  has  written  ? 

7.  Can  he  copy  ? 

Can  he  transfer  print  into  writing  ? 
Can  he  write  numerals  ? 

8.  Can  he  read  aloud  ? 

Can  he  write  from  dictation  ? 

9.  Can  he  name  objects,  letters,  or  numerals  ? 


186  NERVOUS  DISEASES 


APRAXIA 

The  term  apraxia  should  be  restricted  to  that  condition  in 
which  a  patient,  "without  obvious  motor,  sensory,  or  mental 
impairment,  is  unable  to  appry  to  its  proper  use  an  object 
which  he  can  name  and  whose  uses  and  characteristics  he  is 
able  to  describe.     It  is  a  psycho-motor  defect. 

The  term  has  been  loosely  applied  to  cases  in  which 
impairment  of  intelligence  and  obvious  mental  defect  have 
been  present ;  but  pure  cases  have  been  described  in  lesions 
involving  the  frontal  lobes  and  anterior  part  of  the  corpus 
callosum. 


Chapter   III 
VASCULAR    LESIONS 

Arterio- sclerosis  is  a  condition  in  which  there  is  weakening 
of  the  muscular  and  elastic  tissues  of  the  arteries  with  com- 
pensatory fibrous  thickening  of  the  intima.  These  changes 
may  be  general  or  local,  and  render  the  blood-vessel  rigid, 
calcareous,  and  tortuous.  The  lumen  of  the  artery  is 
narrowed. 

Etiology.  Arterial  degeneration  is  an  invariable  sign  of 
senile  decay,  and  is  therefore  found  as  a  natural  phenomenon 
of  advancing  years,  but  in  certain  families  a  tendency  to 
early  vascular  degeneration  is  observed.  Under  fifty  years 
of  age  it  is  more  frequent  in  males  than  in  females,  but  after 
this  age  the  sexes  are  equally  affected.  Certain  diseases — 
such  as  gout,  rheumatism,  and  arthritis— favour  its  develop- 
ment. It  is  a  secondary  effect  of  renal  disease.  The 
toxic  causes  of  vascular  degeneration  are  mainly  alcohol 
and  lead  poisoning.  Meat  eaters  are  more  liable  to  it  than 
those  who  refrain  from  this  food.  It  is  especially  prone 
to  occur  in  those  whose  occupation  requires  continuous 
strain  or  exertion. 

Syphilis  is  one  of  the  chief  causes  of  arterial  degeneration. 
It  may  affect  the  vessels  either  generally  or  locally,  the 
commonest  form  being  a  localised  thickening  of  the  inner 
coat — syphilitic    endarteritis.      Degenerative    changes   may 


DISEASES  OF  THE  BRAIN  187 

also  occur  in  the  muscular  fibres  and  in  the  elastic  lamina, 
which  becomes  split  up  and  separated  by  a  growth  of  new 
cells.  In  more  acute  cases,  periarteritis  with  leucocyte  infil- 
tration is  present,  and  in  the  larger  vessels  the  vasa  vasorum 
may  become  obliterated,  leading  to  further  degeneration. 
There  is  little  tendency  towards  calcification  of  the  arteries. 

Cerebral  lesions  of  vascular  origin  are  produced  by  (1) 
a  cutting  off  or  diminution  of  the  blood  supply,  and  (2)  the 
rupture  of  an  artery. 

The  ultimate  effect  of  vascular  disease  upon  the  cerebral 
tissues  depends  upon  two  factors  :  first,  the  degree  of  the 
vascular  changes,  and  secondly,  the  state  of  the  cardio- 
vascular system. 

In  every  case  of  vascular  disease  there  is— at  least  in 
theory — a  state  of  arterial  blood  pressure  at  which  the 
circulation  may  be  maintained  without  the  occurrence  of 
either  thrombosis  or  hemorrhage. 

In  general  terms,  the  greater  degrees  of  vascular  degener- 
ation, if  associated  with  a  low  blood  pressure,  favour  throm- 
bosis, or  blocking  of  an  artery ;  and  if  the  blood  pressure  is 
high,  rupture  of  an  artery  or  hemorrhage. 

Vascular  lesions  occurring  in  infancy  and  childhood  differ 
from  those  of  later  life  both  in  histological  features  and  clinical 
characteristics.  They  are  therefore  described  separately  in 
the  chapter  on  the  Cerebral  Paralyses  of  Infancy  and  Child- 
hood (p.  211). 

In  adults  the  cutting  off  of  the  blood  supply  may  be 
either  partial  or  complete. 

PARTIAL  OCCLUSION  OR    EVASCULARISATION 

The  pathological  condition  underlying  this  is  widespread 
vascular  degeneration.  The  arteries  show  extensive  arterio- 
sclerosis, and  on  examining  the  brain  even  the  arterioles  are 
seen  to  stand  out  prominently.  There  is  neither  softening  nor 
hemorrhage,  but  occasionally  the  perivascular  tissue  is  faintly 
tinged.  Round  the  vessels  some  rarefaction  of  the  cerebral 
tissues  or  a  definite  increase  of  the  neuroglia  is  observed. 

The  nerve  elements,  cells  as  well  as  fibres,  show  degener- 
ative changes.  The  degeneration  of  the  nerve  fibres  is 
relatively  greater  than  that  of   the    cells,  and   is    specially 


188  NERVOUS  DISEASES 

conspicuous  in  the  region  of  the  hasal  ganglia.  These 
changes  are  probably  brought  about  by  a  failure  of  nutrition 
acting  directly  upon  the  nerve  fibres,  and  indirectly  through 
their  cells  of  origin.  The  cell  changes  consist  of  ehroma- 
tolysis  and  atrophy  (Buzzard  and  Barnes  '). 

Symptoms.  There  is  a  gradual  and  progressive  mental 
impairment,  with  loss  of  emotional  control.  As  time  goes  on 
the  facial  expression  becomes  more  or  less  fixed,  resembling 
that  of  paralysis  agitans.  General  spasticity  slowly  develops 
in  association  with  muscular  weakness  of  general  distribution 
and  slight  degree.  The  deep  reflexes  are  exaggerated,  but 
only  in  the  later  stages  do  definite  signs  of  pyramidal 
degeneration  show  themselves  in  extensor  responses  and 
ankle  clonus. 

The  differential  diagnosis  has  to  be  made  from  paralysis 
agitans  without  tremor,  and  pseudo-bulbar  palsy.  In  the 
early  stages  the  facial  expression,  and  the  slight  degree  of 
spasticity  combined  with  muscular  weakness,  present  a  super- 
ficial resemblance  to  paralysis  agitans ;  but  in  paralysis 
agitans,  mental  impairment  and  emotional  instability  are  not 
found.  In  the  later  stages  the  extensor  plantar  responses 
put  the  diagnosis  of  paralysis  agitans  out  of  court. 

It  differs  from  pseudo-bulbar  palsy  by  the  absence  of  a 
history  of  slight  '  strokes,'  by  its  progressive  character,  its 
symmetrical  distribution,  the  relatively  slight  degree  of 
muscular  weakness  proportionate  to  the  spasticity,  and  by 
the  postponement  of  organic  changes  in  the  reflexes  until 
the  late  stages. 

Complete  occlusion.  This  may  be  due  to:  (1)  Throm- 
bosis, which  consists  of  the  formation  of  a  clot  in  situ 
obstructing  the  blood  stream,  and  finally  occluding  the 
artery.  (2)  Embolism,  or  the  blocking  of  a  vessel  by  a 
clot  carried  from  a  distance. 

CEREBRAL   THROMBOSIS 

This  may  arise  from  :  (a)  Vascular  degeneration,  either 
syphilitic  or  atheromatous.  Syphilis  is  the  common  cause 
in  younger  people  and  between  the  ages  of  thirty  and  forty 

1  F.  Buzzard  and  Barnes,  Review  of  Neurology,  1909. 


DISEASES  OF  THE  BKAIN  180 

years,  and  atheromatous  degeneration  from  the  age  of  fifty- 
five  onwards.  (b)  Enfeebled  circulation  with  low  blood 
pressure  in  senile  cases,  and  in  cases  of  cardiac  failure, 
(c)  Abnormal  states  of  the  blood  favourable  to  clotting,  such 
as  ansemia  and  puerperal  conditions,  (d)  Combinations  of  the 
above  causes,  (e)  Pressure  on  blood-vessels  from  without, 
obstructing  the  flow  of  blood — as  in  tumours,  gummata,  &c. 

In  a  series  of  25  cases  of  thrombotic  softening  sub- 
mitted to  post-mortem  examination,  20  showed  well-marked 
arterial  disease ;  of  these,  9  had  granular  kidneys,  8  were 
syphilitic,  and  3  were  alcoholic.  In  5  the  blood-vessels  were 
stated  to  be  normal ;  in  2  of  these  thrombosis  was  associated 
with  acute  rheumatism,  and  in  1  with  the  puerperium. 

Situation  of  cerebral  softening.  Some  regions  corre- 
sponding to  areas  of  arterial  distribution  are  more  frequently 
affected  by  softening  than  others.  A  perusal  of  the  cases 
submitted  to  post-mortem  examination  showed  that  the  basal 
ganglia,  more  especially  the  lenticular  nucleus,  with  the 
closely  related  internal  capsule,  were  the  structures  most 
frequently  involved.  Next  in  frequency  came  the  caudate 
nucleus,  optic  thalamus,  and  adjacent  parts  of  the  centrum 
ovale  and  corona  radiata.  The  frontal  or  anterior  segment 
of  the  centrum  ovale  was  more  commonly  involved  than  the 
occipital  or  posterior  segment.  In  marked  contradistinction 
to  what  was  seen  in  hemorrhage,  the  temporo- sphenoidal 
lobe  was  frequently  the  seat  of  thrombotic  softening.  Next 
in  order  of  frequency  were  the  pons,  cerebellum,  midbrain, 
and  the  corpus  callosum. 

The  extent  of  the  softened  area  depends  upon  the  size  and 
distribution  of  the  affected  vessel,  and  the  area  of  collateral 
circulation.  When  softening  occurs  in  non-vascular  parts  of 
the  brain,  there  is  simple  necrosis  of  the  white  matter  with 
formation  of  fatty  debris  ;  if  in  a  vascular  area,  it  is  red  in 
colour  owing  to  the  regurgitation  of  blood  into  the  softened 
zone.  Yellow  softening  is  merely  a  later  stage  of  red  soften- 
ing, in  which  changes  have  taken  place  in  the  blood  pigment. 
Where  the  vascular  occlusion  has  been  only  partial,  or  after 
the  establishment  of  collateral  circulation,  a  slow  degeneration 
of  the  myeline  sheaths  occurs  with  secondary  proliferation  of 
the  neuroglia.  This  has  been  spoken  of  as  grey  softening, 
but  is  really  sclerosis. 


100  NERVOUS  DISEASES 

Symptoms  of  thrombosis.  General  symptoms  due  to  the 
underlying  arterio-sclerosis  may  be  present  for  some  months 
before  the  stroke.  These  are  alterations  in  temperament  and 
mental  condition,  failure  of  memory,  irritability,  lethargy, 
and  drowsiness,  often  accompanied  by  slight  general  spasticity 
and  articulator}7  difficulty.  Focal  symptoms — such  as  numb- 
ness or  transitory  paresis — are  usually  referred  to  the  region 
of  the  body  subsequently  affected  by  the  stroke,  and  are 
dependent  upon  minute  thrombotic  occlusions. 

Degenerative  vascular  changes  and  hemorrhages  in  the 
fundus  oculi  frequently  precede  the  onset  of  the  cerebral 
thrombosis  by  some  months. 

The  premonitory  symptoms  may  usher  in  the  stroke  by  a 
few  hours,  and  consist  of  headache,  giddiness,  mental  dullness, 
and  an  increasing  sleepiness  and  lethargy. 

The  onset  of  thrombosis  may  be  (a)  gradual,  (b)  sudden 
without  impairment  of  consciousness,  or  (c)  sudden  with  loss 
of  consciousness. 

(a)  The  onset  may  be  gradual,  unattended  by  any  loss  of 
consciousness,  and  consist  merely  of  an  increase  of  the  paretic 
or  paresthetic  premonitory  symptoms,  already  mentioned. 

(b)  In  a  large  number  of  cases  the  onset  of  paralytic 
symptoms  is  more  or  less  sudden  and  incomplete  with 
preservation  of  consciousness,  but  followed  some  hours  later 
by  a  further  increase  of  paralysis  associated  with  loss  of 
consciousness.  Instances  of  this  type  of  thrombosis  are 
seen  in  those  cases  in  which  during  the  evening  a  patient 
complains  of  unilateral  numbness  and  paresis,  but  subse- 
quently loses  consciousness,  and  is  found  hemiplegic  in 
the  morning. 

(c)  There  are  other  cases,  in  which  the  onset  is  sudden 
with  initial  loss  of  consciousness,  and  attended  sometimes  by 
unilateral  or  bilateral  convulsions. 

The  loss  of  consciousness  in  cases  of  thrombosis  is  not  so 
sudden  or  constant  a  feature  as  in  cerebral  hemorrhage.  In 
thrombosis,  also,  there  is  usually  definite  evidence,  or  a 
history  of  previous  paretic  or  hemiplegic  symptoms,  and 
coma  is  rarely  so  profound  even  when  prolonged.  The 
exception  to  this  is  found  in  those  cases  in  which  large 
vessels,  such  as  the  middle  cerebral  artery,  become  blocked, 
when  delirium  and  restlessness  precede  the  onset  of  coma. 


DISEASES  OF  THE  BRATN  191 

The  focal  symptoms  are  those  arising  from  occlusion  of 
particular  arteries. 

(a)  Internal  carotid  artery.  The  symptoms  are  usually 
profound  and  extensive,  causing  hemiplegia,  coma,  and 
death. 

(b)  Middle  cerebral  artery.  If  the  artery  is  blocked  at 
its  source  on  the  left  side,  motor  and  sensory  aphasia,  with 
complete  right-sided  hemiplegia  and  hemianesthesia,  ensue. 
If,  however,  thrombosis  is  limited  to  the  first  branch,  there 
are  motor  aphasia  and  paralysis  of  the  face  and  tongue,  with 
weakness  usually  of  the  arm ;  and  occlusion  of  the  second 
branch  produces  the  brachio-facial  type  of  hemiplegia.  If 
the  Sylvian  branch  is  blocked  beyond  the  fissure  of  Rolando, 
slight  hemiplegia  with  a  well-defined  word-blindness  and 
hemianopsia  are  present,  and  sometimes  word-deafness. 

(c)  Posterior  cerebral  artery.  Occlusion  of  this  artery 
is  followed  by  sensory  symptoms,  chiefly  hemianopsia  and 
hemianesthesia,  and  sometimes  by  tremor  of  the  limbs  on 
the  opposite  side. 

(d)  Basilar  artery.  The  symptoms  of  occlusion  of  this 
vessel  are  bilateral  paralysis  of  the  limbs  and  articulator}' 
paralysis.  If  the  lesion  is  towards  the  termination  of  the 
artery,  there  is  a  third  nerve  paralysis  with  crossed  hemi- 
plegia ;  if  the  occlusion  is  in  the  middle  portion  of  the 
artery,  the  symptoms  are  facial  paralysis,  palsy  of  the  fifth 
nerve,  and  of  the  lateral  conjugate  movements  of  the  eyes. 

(e)  Although  the  area  supplied  by  the  anterior  cerebral 
artery  is  frequently  the  seat  of  softening,  symptoms  resulting 
from  its  occlusion  are  not  distinctive,  but  are  probably 
psychical  in  character,  and  of  the  nature  of  progressive 
dementia. 

Prognosis  and  course.  In  syphilitic  cases  the  prognosis, 
both  as  regards  the  stroke  and  the  subsequent  duration  of  life, 
is  favourable ;  in  our  series  life  was  prolonged  from  two  to  ten 
years.  In  atheromatous  cases  the  duration  of  life  is  less 
likely  to  exceed  two  years,  but  may  be  prolonged  three  or 
even  five  years.  In  both  series  the  existence  of  renal  disease 
or  hypertrophy  of  the  heart  renders  the  prognosis  much  less 
favourable,  the  subsequent  duration  of  life  being  rarely  more 
than  one  year.  Recovery  from  the  immediate  effects  of 
the   stroke  is  good,  except  in  cases  where  consciousness  is 


192  NER  Vol's  DISEASES 

lost  for  more  than  forty-eight  hours,  although  prolonged 
unconsciousness  is  not  so  unfavourable  as  in  hemorrhage. 

If  no  return  of  motor  power  occurs  within  two  or  three 
weeks  of  the  onset  of  paralysis,  the  outlook  as  regards 
recovery  from  the  paralysis  is  unfavourable,  as  softening  in 
the  area  of  the  thrombosed  artery  ensues  with  permanent 
paralysis,  rigidity,  and  contracture. 

Treatment,  (a)  Immediate  treatment.  In  the  syphilitic 
cases  stimulation  is  indicated  if  the  blood  pressure  is  not 
high.  If  there  is  a  previous  history  of  thrombotic  attacks, 
stimulants  ought  to  be  prescribed  with  care,  and  their 
administration  guided  by  the  state  of  the  blood  pressure,  as 
in  such  cases  the  vessels  at  the  seat  of  old  thromboses  are 
liable  to  rupture  from  sudden  increase  of  arterial  tension. 

As  soon  as  the  diagnosis  is  made,  a  course  of  anti- 
syphilitic  treatment  by  mercury  and  the  iodides  should  be 
commenced. 

In  the  atheromatous  cases,  if  the  blood  pressure  is  low, 
and  there  is  no  history  of  previous  thrombosis,  stimulants 
may  be  given. 

If  there  is  associated  renal  disease  and  cardiac  hyper- 
trophy, stimulants  should  be  avoided  entirely,  or  only  pre- 
scribed in  the  form  of  mild  diffusible  drugs.  In  these  cases 
the  injudicious  giving  of  stimulants,  such  as  alcohol,  strychnine, 
and  digitalis,  has  resulted  in  fatal  cerebral  hemorrhage.  The 
indiscriminate  use  of  purgatives  is  to  be  avoided  in  most 
cases. 

(b)  After  treatment.  Attention  should  be  devoted  to  the 
condition  of  the  bladder,  the  mouth,  lips,  and  tongue ;  and 
to  the  skin,  as  bed-sores  may  form.  The  diet  should  be 
light  and  nutritious.  As  regards  the  paralysis,  passive 
movements  should  be  prescribed  several  times  daily  after  the 
first  week.  The  tendency  to  rigidity  and  flexor  contractures 
is  overcome  by  passive  movements  and  massage,  combined 
with  electrical  applications  to  the  extensor  groups  of  muscles. 
The  patient  should  also  be  encouraged  to  move  the  limbs, 
and  when  possible  to  re-educate  the  hand  in  the  finer  move- 
ments of  writing,  &c.  Adhesions  at  the  joints  may  require 
to  be  broken  down  under  anaesthesia,  and  inflammatory 
changes  are  prevented  or  minimised  by  the  local  application 
of  hot-air  or  radiant-heat  baths. 


DISEASES  OF  THE  BRATN  193 

CEREBRAL    EMBOLISM 

In  embolism  the  obstruction  of  the  blood  stream  is 
produced  by  a  clot  or  foreign  body  carried  from  a  distant 
part.  Such  a  clot  must  of  necessity  be  derived  from  some 
portion  of  the  circulatory  apparatus  between  the  lungs  and 
its  place  of  lodgement. 

Emboli  are  usually  derived  from  the  heart,  in  consequence 
either  of  a  cardiac  clot  or  an  endocardial  lesion.  Occasionally 
a  portion  of  a  cerebral  thrombus  is  detached  and  lodged  in  a 
distal  blood-vessel.  Septic  conditions  may  also  give  rise  to 
emboli,  especially  in  the  small  arteries,  and  lead  to  acute 
multiple  softenings,  which  may  be  purulent. 

In  embolism  an  acute  oedema  of  the  cerebral  tissue 
around  the  infarct  takes  place,  but  the  later  changes  are 
identical  with  those  described  under  thrombosis. 

It  usually  occurs  in  young  people,  either' during  an  attack 
of  acute  endocarditis,  or  may  supervene  at  any  time 
afterwards  from  the  detachment  of  a  fragment  of  fibrous 
material. 

Symptoms,  The  onset  is  sudden,  and  is  attended  by 
loss  of  consciousness,  which  is  rarely  profound,  more  or  less 
transient,  and  depends  in  most  cases  upon  the  suddenness  of 
the  shock.  Convulsions  frequently  occur.  Paralysis  may 
or  may  not  be  present,  depending  upon  the  situation  of  the 
blocked  artery.  Usually  a  small  artery  is  affected,  so  that 
paralysis  need  not  be  extensive.  On  the  other  hand,  a  large 
artery  may  be  blocked,  with  extensive  paralysis ;  this,  however, 
is  a  rarer  condition.  The  extent  and  severity  of  the  paralysis 
may  increase  slightly  within  twelve  hours  after  the  onset  of 
the  symptoms,  owing  to  the  local  cedematous  conditions 
around  the  embolic  area.  Focal  symptoms  depend  upon  the 
vessel  occluded,  as  described  under  thrombosis  (p.  191). 

The  temperature  depends  mainly  upon  the  associated 
causal  condition,  but  may  be  raised  for  a  short  time  after  the 
stroke ;  the  respirations  are  not  materially  altered.  Examin- 
ation of  the  heart  demonstrates  the  presence  of  valvular 
disease  or  an  active  endocarditis.  The  blood-vessels  are 
normal,  but  evidence  of  infarcts  in  other  organs — such  as  the 
spleen  and  the  kidneys— may  be  present. 

The  prognosis  depends  upon  the  causing   condition.     If 

13 


194  NERVOUS  DISEASES 

occurring  during  the  course  of  an  acute  endocarditis,  ulcerative 
valvular,  disease,  or  pyaemia,  the  prognosis  is  unfavourable. 
In  the  absence  of  acute  maladies,  such  as  the  above,  the 
outlook  is  favourable,  as  recovery  may  take  place,  especially 
in  those  cases  in  which  little  or  no  paralysis  is  present. 

Treatment.  The  general  principles  are  the  same  as  for 
thrombosis,  more  particularly  as  regards  the  administration 
of  stimulants.  Rest  in  bed,  the  avoidance  of  all  unnecessary 
movements,  and  attention  to  the  bladder  and  bowels,  are 
important.  The  general  treatment  is  also  to  a  large  extent 
that  of  the  causing  condition. 

CEREBRAL  HEMORRHAGE 

Two  conditions  are  accessory  to  the  production  of  cerebral 
hemorrhage  :  (a)  a  structural  weakening  of  the  blood-vessel, 
and  (b)  a  blood  pressure  sufficiently  high  to  rupture  the 
weakened  vessel.  The  latter  condition  is  the  determining 
factor,  and,  as  far  as  our  knowledge  goes,  there  is  no  state  of 
continued  high  blood  pressure  without  definite  pathological 
changes  occurring  in  the  arterial  vascular  system.  But 
however  essential  a  high  blood  pressure  may  be  in  the  pro- 
duction of  cerebral  hemorrhage,  rupture  of  a  normal  artery, 
whose  walls  are  well  supported,  is  wellnigh  impossible. 
Thus  it  comes  about  that  in  the  study  of  cases  of  cerebral 
hemorrhage,  evidence  of  arterial  degeneration  is  never  lacking. 

The  weakening  of  the  artery  is  due  to  structural  changes 
in  its  walls,  leading  to  loss  of  elasticity.  Where  such  a 
condition  exists,  a  temporary  increase  in  the  blood  pressure 
may  of  itself  suffice  to  cause  a  rupture.  This  increase  is,  as 
a  rule,  abrupt  and  profound,  and  may  be  due  to  sudden 
emotional  states  or  excessive  physical  strain.  Extensive 
vascular  disease,  with  a  corresponding  and  compensatory 
hypertrophy  of  the  heart,  rarely,  if  ever,  results  in  cerebral 
hemorrhage  without  the  presence  of  the  exciting  causes  just 
mentioned. 

The  flow  of  the  blood  stream  through  the  narrowed  and 
roughened  arterial  lumen  is  retarded,  and  may  eventually  be 
arrested.  Such  a  condition  is  most  liable  to  occur  in  the 
smaller  arterioles  or  end-arteries,  and  leads  to  malnutrition 
and    softening    of    the   surrounding     brain    tissue.     To  the 


DISEASES  OF  THE  BRAIN  195 

original  weakness  of  the  vascular  system  is  added  the  addi- 
tional weakness  of  the  supporting  tissues — a  combination 
which  favours  the  occurrence  of  rupture  and  hemorrhagic 
effusion.  In  such  cases  the  blood  pressure,  which  is  alone 
not  sufficient  to  cause  rupture  of  a  well-supported  though 
degenerated  artery,  may  readily  induce  it  in  one  surrounded 
by  badly  nourished  or  softened  tissue.  For  example,  a 
patient  with  atheromatous  blood-vessels  and  hypertrophy  of 
the  heart  is  laid  aside  by  illness.  In  consequence  the  blood 
pressure  falls,  and  indefinite  symptoms  of  minute  vascular 
thromboses — such  as  paresthesia,  paresis,  or  mental  altera- 
tions— present  themselves.  As  the  general  condition  improves 
the  thrombotic  symptoms  pass  away,  but  the  blood  pressure 
returns  to  its  original  state,  and  symptoms  of  cerebral 
hemorrhage  may  suddenly  supervene.  In  such  a  case 
no  further  exciting  cause  of  arterial  rupture  may  be 
detected. 

We  find  that  out  of  22  cases  of  cerebral  hemorrhage 
submitted  to  post-mortem  examination,  either  atheromatous 
or  syphilitic  disease  of  the  cerebral  blood-vessels  was  present 
in  all;  9  (or  40  per  cent.)  were  cases  of  primary  hemorrhage, 
and  all  had  granular  kidneys  and  hypertrophy  of  the  heart ; 
13  (or  59  per  cent.)  had  a  history  of  previous  thrombotic 
occlusions.  Of  those,  five  had  syphilitic  arterial  disease 
without  cardiac  hypertrophy  or  renal  disease,  and  the 
remainder  were  cases  of  atheromatous  degeneration. 

Site  of  hemorrhage.  Hemorrhage  may  occur  in  any 
part  of  the  brain,  but  there  are  certain  situations  where  it  is 
more  common.  These  are  :  the  internal  capsule,  the  lenticular 
nucleus,  and  the  optic  thalamus.  The  arteries  which  rupture 
in  this  position  are  the  lenticulo-optic  and  lenticulo-striate 
branches  of  the  middle  cerebral  artery. 

Next  in  frequency  comes  the  centrum  ovale — -the  seat  of 
hemorrhage  being  more  common  in  the  frontal  segment,  from 
rupture  of  the  anterior  cerebral  artery,  than  in  the  occipital 
segment.  When  hemorrhage  occurs  into  either  the  basal 
ganglia  or  the  adjacent  centrum  ovale,  it  is  prone  to  rupture 
into  the  lateral  ventricle.  Hemorrhage  into  the  ventricle, 
therefore,  is  not  uncommon. 

Hemorrhage  into  the  temporo-sphenoidal  lobe,  cerebellum, 
corpus  callosum,  and  midbrain  is  rare. 

13  * 


196  NERVOUS  DISEASES 

Meningeal  hemorrhage  (non-traumatic)  is  found  in  associa- 
tion with  acute  rheumatism,  endocarditis,  and  the  puerperiuin, 
the  arteries  being  healthy. 

Effects  of  hemorrhage.  In  most  cases  of  cerebral 
hemorrhage  blood  is  effused  locally  into  the  brain  substance, 
the  subdural  space,  or  the  ventricles,  where  it  forms  a  clot, 
which  acts  like  a  foreign  body  or  tumour  within  the  brain. 
The  experiments  of  Leonard  Hill1  have  shown  that  such  a 
clot  may  cause  translocation  of  the  brain  as  a  whole,  while 
producing  a  local  compression  of  that  part  of  the  brain  into 
which  the  hemorrhage  has  taken  place. 

Hill's  observations  point  to  the  symptoms  of  cerebral 
hemorrhage  being  those  of  compression  produced  by  cerebral 
anaemia.  The  first  effect  of  a  hemorrhage  is  to  obliterate 
the  capillaries  and  veins  in  its  immediate  neighbourhood,  and 
to  raise  the  intracranial  pressure  there  to  the  level  of  the 
arterial  pressure.  In  the  adjacent  parts,  where  the  blood- 
vessels are  not  completely  obliterated,  the  pressure  will  be 
less  high,  and  in  the  most  distant  parts  of  the  brain — such  as 
the  cerebellum  and  bulb — the  intracranial  pressure  may  be 
normal.  The  major  symptoms  of  apoplexy  will  therefore 
only  arise  when  the  bulbar  capillaries  are  obliterated.  For 
this  reason  a  comparatively  small  subtentorial  hemorrhage 
will  prove  fatal,  while  a  much  larger  effusion  in  the  cerebral 
chamber  is  necessary  to  cause  death. 

Hill  has  pointed  out  that  the  cause  of  death  in  cerebral 
compression  is  bulbar  anaemia,  with  (1)  primaiy  failure  of 
respiration  and  secondary  failure  of  the  heart  and  vaso- 
motor mechanism  ;  or  (2)  primary  failure  of  the  vaso-motor 
centre  with  secondary  failure  of  the  respiration  and  the  heart. 

Should  the  patient  survive  the  immediate  effects  of  the 
hemorrhage,  there  will  be  in  the  cranium  a  localised  foreign 
body,  destroying  the  functions  of  a  certain  portion  of  the 
brain.  Surrounding  this  is  a  compressed  zone  in  which  the 
circulation  has  ceased,  and  outside  this  a  less  compressed 
area  in  which  the  circulation  continues  with  difficulty.  Any 
increase  of  the  arterial  pressure  will,  under  these  conditions, 
increase  the  compression,  and  so  lead  to  anaemia  of  other 
vascular  areas.     This  point  is  of  great  practical  importance 

1  Leonard  Hill,  The  Cerebral  Circulation,  1890. 


DISEASES  OF  THE   BRAIN  107 

in  the  treatment  of  cerebral  hemorrhage ;  as  lowering  of  the 
arterial  pressure  by  means  of  venesection  or  local  depletion 
would  seem  to  be  the  procedure  indicated  on  physiological 
grounds. 

Etiology.  Cerebral  hemorrhage  is  more  common  in  men 
than  in  women,  their  occupation  and  habits  predisposing 
them  to  vascular  degeneration.  We  found  it  most  frecpaent 
between  the  ages  of  forty-seven  and  fifty-five.  In  those  with 
a  syphilitic  history  the  age  at  onset  was  a  few  years  earlier, 
probably  owing  to  the  presence  of  previous  thrombotic 
softenings.  In  the  older  cases  the  underlying  atheroma 
had  prepared  the  way  by  antecedent  softening. 

The  predisposing  causes  are  :  cerebral  softening,  atheroma, 
renal  disease,  and  cardiac  hypertrophy ;  syphilitic  endar- 
teritis;  alcohol,  lead,  and  gout;  and  blood  states — such  as 
purpura,  pernicious  anaemia,  and  leucocythemia. 

The  exciting  factors  are  :  physical  efforts— such  as 
straining  at  stool,  coughing,  vomiting,  or  hurrying ;  and 
emotional  states — such  as  rage,  fright,  anger,  shock,  and 
anxiety. 

Symptoms.  (a)  Premonitory  symptoms.  A  sense  of 
fullness  and  throbbing  in  the  head,  vertigo,  headache,  and 
epistaxis  are  occasionally  met  with  as  premonitory  symptoms 
of  impending  hemorrhage.  These  ought  to  be  distinguished 
from  the  slight  mental  impairment,  paresis,  spasticity  of  the 
legs,  and  dysarthria,  which  are  common  antecedent  symptoms 
of  cerebral  thrombosis  and  hemorrhage,  and  are  due  to  the 
underlying  arterio-sclerosis. 

(b)  The  mode  of  onset  differs  according  to  the  situation 
of  the  rupture,  and  to  the  severity  and  rapidity  of  the 
effusion ;  but  as  a  general  rule  consciousness  is  lost  at  once  if 
the  hemorrhage  is  sudden  and  extensive.  Convulsions,  often 
one-sided,  may  usher  in  an  attack  when  the  lesion  is 
meningeal  or  cortical ;  but  larger  hemorrhages  within  the 
brain  may  also  be  attended  by  generalised  convulsion. 

(c)  The  general  symptoms  vary  according  to  the  size  and 
character  of  the  effusion.  If  the  effusion  is  small  and 
circumscribed,  the  patient  is  suddenly  seized  with  a  feeling 
of  faintness  or  weakness,  the  speech  becomes  slurred,  and 
paresis  or  numbness  may  develop  on  one  side  of  the  body. 
He  is  forced  to  sit  down,  or  may  fall,  the  hemiplegic  weakness 


198  NERVOUS  DISEASES 

increases  in  intensity,  dullness  and  drowsiness  supervene, 
consciousness  is  lost,  and  coma  ensues. 

If  the  effusion  is  larger  and  more  rapid  in  onset,  the 
patient  suddenly  falls  down  unconscious,  and  may  remain  so 
for  one  or  more  days.  With  the  recovery  of  consciousness 
the  reflex  actions  of  the  pupil  and  of  swallowing  return,  and 
unilateral  flaccid  paralysis  is  detected. 

On  the  other  hand,  the  onset  of  the  symptoms  may  be  of 
a  slow  and  ingravescent  type.  In  these  cases  the  commence- 
ment of  the  seizure  is  prolonged,  paralysis  gradually  develops, 
and  consciousness,  at  first  impaired,  rapidly  becomes  lost. 
During  the  early  stages  the  paretic  may  be  distinguished  from 
the  non-paralysed  side  b}^  the  exaggeration  of  the  deep  and 
the  absence  of  the  abdominal  reflexes,  and  the  presence  of  an 
extensor  plantar  response.  The  pupil  on  the  hemiplegic  side 
is  usually  the  larger.  The  temperature  is  normal  or  sub- 
normal, the  pulse  slow,  firm,  and  incompressible,  and  the 
respirations  slightly  increased.  The  coma  eventually  deepens, 
the  limbs  on  both  sides  become  flaccid,  the  deep  reflexes 
disappear,  and  finally  the  pupillary  light  reflex  is  lost.  A 
rising  temperature  and  a  rapid,  irregular  pulse,  associated 
with  slowr,  cyclic,  or  Cheyne-Stokes  respiration  and  wide, 
dilated  pupils,  becoming  pin-point  in  size,  complete  the 
clinical  picture. 

When  hemorrhage  ruptures  into  the  ventricle,  the  loss  of 
consciousness  is  sudden  and  complete,  and  may  be  accom- 
panied by  convulsion  and  vomiting.  The  limbs  are  flaccid, 
the  breathing  stertorous,  the  face  flushed,  and  the  cheeks  are 
puffed  out  at  each  expiration.  Coma  is  profound,  and  the 
reflexes  are  lost — the  light-reflex  of  the  pupil  as  well  as  the 
deep  and  superficial  reflexes.  The  breathing  becomes  slow 
and  irregular — 6  to  14.  per  minute ;  often  more  or  less  cyclic, 
three  or  four  respirations  coming  together,  and  followed  by 
a  pause,  or  of  the  Cheyne- Stokes  type.  The  pulse,  at  first 
full  and  slow,  rapidly  becomes  feeble  and  irregular — 120 
to  150  per  minute.  The  pupils,  widely  dilated  at  the  outset, 
become  contracted.  The  temperature  shows  an  initial 
depression,  but  rapidly  rises  to  104°-107°  F.,  and  death  soon 
ensues  without  return  to  consciousness. 

Symptoms  of  compression.  With  the  stroke  the  patient 
falls  down  unconscious,  and  passes  into  a  state  of  coma.     The 


DISEASES  OF  THE  BRATN  199 

muscles  are  relaxed  and  flaccid,  all  reflex  action  is  abolished, 
and  urine  and  fscces  may  be  passed  involuntarily.  The 
breathing  is  laboured  and  stertorous,  sometimes  cyclic  or 
Cheyne- Stokes  in  character.  The  pulse  as  a  rule  is  slow  and 
incompressible.  During  this  stage  it  may  be  impossible  to 
tell  which  is  the  paralysed  side. 

If  the  coma  is  less  deep  the  pupils  react  to  light,  and  the 
reflex  act  of  swallowing  may  be  accomplished.  The  head 
and  eyes  are  turned  to  the  side  of  the  cerebral  lesion  (para- 
lytic deviation),  reflex  action  returns  on  the  non-paralysed 
side,  and  on  raising  the  paralysed  arm  and  leg  they  will  be 
found  to  be  flaccid  and  powerless,  while  those  on  the  non- 
paralyeed  side  do  not  show  the  same  degree  of  flaccidity.  If 
the  patient  remains  in  a  state  of  coma  bed-sores  may  form, 
and  death  occur.  In  most  cases  where  some  recovery  of 
consciousness  occurs  within  the  first  twelve  hours,  there  is 
a  short  period,  lasting  for  one  or  two  days,  in  which  the 
temperature  rises,  the  general  condition  appears  to  be 
worse,  consciousness  is  again  blunted,  and  the  limbs  on  the 
paralysed  side  are  noticed  to  be  rigid.  This  is  '  early 
rigidity,'  and  is  a  transitory  symptom. 

During  the  next  few  days  the  general  condition  improves, 
so  that  the  extent,  degree,  and  quality  of  the  paralysis  become 
defined.  In  cerebral  hemorrhage  the  initial  paralysis  is  in 
excess  of  the  permanent,  owing  to  pressure  of  the  effused 
blood  upon  adjacent  nerve  tracts,  and  indirectly  on  other 
portions  of  the  brain.  These  pressure  effects  are  reduced 
with  the  absorption  of  the  clot. 

Pontine  hemorrhage,  Initial  loss  of  consciousness  is 
sudden  and  profound,  should  the  effusion  be  large.  Vomiting 
arid  convulsions,  usually  bilateral,  are  sometimes  present. 
There  is  complete  flaccid  paralysis  with  abolition  of  the 
reflexes.  The  pupils  are  contracted  to  the  size  of  pin-points. 
Respiration  is  often  markedly  irregular,  and  may  cease  suddenly 
— sometimes  before  the  pulse  stops.  The  temperature,  at  first 
depressed,  rises  rapidly  to  hyperpyrexia,  and  death  may  occur 
within  a  few  hours,  or  even  within  a  few  minutes,  of  the 
onset  of  the  first  symptoms. 

Multiple  hemorrhages  of  small  size  may  also  occur  in  the 
pons.  Their  onset  is  unattended  by  loss  of  consciousness  : 
the  palsy  of  the  limbs  may  be  on  one  or  other,  or  both  sides  : 


200  NERVOUS  DISEASES 

the  articulation  is  defective  and  nasal  in  character,  and 
swallowing  may  be  impaired.  The  picture  presented  in 
these  cases  is  a  form  of  bulbar  palsy,  forming  one  of 
the  types  of  '  pseudo-bulbar  '  paralysis. 

In  addition  to  these  symptoms,  paralysis  or  paresis  of 
several  cranial  nerves  may  be  observed.  The  nerves  commonly 
involved  are  the  Mill,  sixth,  seventh,  ninth,  tenth,  and  twelfth. 
Paralysis  of  the  higher  cranial  nerves  may  be  found  upon  one 
side,  with  crossed  motor  and  sensoiy  paralyses  of  the  body 
and  limbs,  and  in  some  cases  paralysis  of  conjugate  lateral 
movement  of  the  eyes  may  be  noticed. 

Cerebellar  hemorrhage.  Sudden  and  extensive  hemor- 
rhage into  the  cerebellum  is  accompanied  by  unconsciousness, 
but  usually  unattended  by  hemiplegic  weakness  unless  the 
pons  is  also  affected.  The  vomiting  is  specially  characteristic, 
and  often  persistent.  Skew  deviation  of  the  eyeballs,  and  a 
tendency  to  rotate  to  the  side  of  the  lesion,  may  be  present. 
Cerebellar  hemorrhage  maj^  rupture  into  the  fourth  ventricle 
and  cause  death  within  a  few  hours.  In  these  cases  sugar  is 
present  in  the  urine  without  the  acetone  reaction. 

Small  hemorrhages  limited  to  the  cerebellum  are  un- 
accompanied by  loss  of  consciousness.  The  patient  is  seized 
with  vertigo  and  vomiting,  which  are  aggravated  with  every 
movement  of  the  head.  Skew  deviation  of  the  eyes  may  be  a 
transient  symptom.  If  the  lesion  is  limited  to  one  lateral 
lobe,  a  staggering  gait  with  a  tendency  to  fall  to  the  side  of 
the  lesion  and  coarse  nystagmoid  jerkings  of  the  eyes  on 
conjugate  movement  towards  the  affected  side  are  observed. 
Ataxia  of  the  limbs  on  the  side  of  the  lesion  is  well 
marked. 

If  the  hemorrhage  takes  place  into  the  middle  lobe, 
opisthotonos  may  occur ;  but  in  the  later  stages  the  gait  is 
unsteady,  with  a  tendency  for  the  patient  to  fall  forwards  or 
backwards.  Coarse  nystagmus  is  also  present  on  looking  to 
either  side,  and  ataxia  -  especially  of  the  trunk  and  lower 
limbs. 

Meningeal  hemorrhage.  This  variety  of  intracranial 
hemorrhage  rna,y  be  due  either  to  traumatic  or  non-traumatic 
causes.  Of  the  latter,  acute  rheumatism,  endocarditis,  and 
the  puerperium  are  the  most  common.  Vascular  degeneration 
is  a  rare  cause  of  hemorrhage  in  this  situation. 


DISEASES  OF  THE  BRAIN  201 

The  patient  usually  complains  of  sudden  and  severe  pain 
in  the  head,  calls  out,  and  falls  down  convulsed.  Sometimes 
the  convulsion  is  limited  to  one  side  without  loss  of  con- 
sciousness ;  but  if  the  effusion  is  extensive  consciousness  is 
lost,  and  coma  is  induced,  which  may  persist,  deepen,  and 
rapidly  prove  fatal  with  respiratory  and  cardiac  failure  and 
hyperpyrexia. 

If  the  patient  survives  the  persistence  of  coma  for  more 
than  twenty- four  hours,  the  chances  of  recovery  increase,  as 
the  hemorrhage  may  be  arrested  by  the  pressure  of  the  clot 
upon  the  ruptured  vessel. 

In  other  cases  the  initial  hemorrhage  may  be  circum- 
scribed, so  that  the  symptoms  consist  only  of  slight  hemiparesis 
of  the  limbs  opposite  the  lesion.  An  effort  of  exertion,  or 
alcoholic  stimulant  taken  at  this  time,  may  favour  the 
recurrence  of  hemorrhage  with  rapid  loss  of  consciousness. 

In  many  cases  delirium,  mental  confusion,  and  dementia 
are  also  present. 

Hemorrhage  into  the  basal  meninges  proves  rapidly  fatal. 
The  pupils  become  pin-point,  coma  deepens,  the  temperature 
rises  to  hyperpyrexia,  the  pulse  is  rapid  and  feeble,  and  death 
results  from  respiratory  failure. 

In  traumatic  cases,  if  the  hemorrhage  is  extradural  and 
operation  for  its  removal  adopted  early,  the  outlook  is  good  ; 
put  if  the  hemorrhage  is  primarily  basal,  death  occurs  in 
a  few  hours. 

In  a  few  cases  where  the  effusion  is  limited  by  adhesions 
between  the  dura  mater  and  the  skull,  the  prognosis  is  more 
favourable,  especially  if  operation  is  undertaken  for  the 
removal  of  the  clot. 

The  outlook  in  cases  of  intradural  hemorrhage  is  most 
unfavourable.  In  basal  cases  and  in  those  arising  from 
rupture  of  an  aneurism  the  fatal  termination  is  rapid. 
The  early  recognition  of  ingravescent  hemorrhage,  whether 
arterial  or  venous,  and  the  adoption  of  surgical  measures, 
may  save  life. 

Prognosis.  A  prognosis  has  to  be  given  upon  three 
points :  — 

1.  The  immediate  prognosis  as  regards  life.  The  follow- 
ing are  unfavourable  features  in  a  case  of  apoplexy :  (a) 
Deep  coma  prolonged  over  twenty-four  hours  ;   (b)  cyclic  or 


202  NERVOUS  DISEASES 

Cheyne- Stokes  respiration  persisting  for  more  than  six  or 
eight  .hoars  ;  (c)  severe  initial  depression  of  temperature, 
or  a  rapid  pyrexial  rise  after  an  initial  depression  ;  (d)  a 
bilateral  distribution  of  the  paralysis ;  (e)  the  presence  of 
complications — such  as  pulmonary  congestion,  severe  renal 
or  cardiac  disease,  or  the  occurrence  of  bed-sores. 

2.  Future  prospect  of  life.  This  is  always  less  favour- 
able than  in  cases  of  thrombosis.  If  there  has  been  a  history 
of  antecedent  thrombotic  attacks,  especially  when  associated 
with  renal  disease  and  hypertrophy  of  the  heart,  the  duration 
of  life  rarely  exceeds  one  year,  and  may  be  shorter.  If  the 
stroke  has  been  due  to  a  violent  strain  or  emotion  without 
previous  symptoms  of  cerebral  vascular  disease,  careful  regula- 
tion of  the  patient's  habits,  and  especially  of  the  blood 
pressure,  renders  the  prognosis  less  grave,  and  life  may  be 
prolonged  for  two  or  two  and  a  half  years. 

3.  Becovery  from  paralysis.  As  a  general  rule,  those 
portions  of  the  body  in  which  movement  returns  within  one 
month  of  the  stroke  will  recover,  provided  that  the  occurrence 
of  adhesions  and  joint  changes  is  prevented ;  but  when  no 
movement  has  returned  within  three  months  of  the  stroke 
the  paralysis  is  likely  to  be  permanent. 

Treatment.  The  treatment  of  cerebral  hemorrhage  is 
directed  towards  saving  the  patient's  life,  which  is  threatened 
by  cerebral  compression  and  anaemia  of  the  bulbar  centres. 
The  symptoms  of  compression  are,  as  already  explained,  due 
to  increased  intracranial  pressure  from  the  presence  of  the 
hemorrhagic  clot,  acting  as  a  foreign  body  within  the 
skull. 

The  physician  has  two  objects  before  him  :  first,  the 
arrest  of  the  hemorrhage,  and  secondly,  the  relief  of 
compression. 

The  methods  of  arresting  hemorrhage  are  :  complete  rest, 
allaying  the  action  of  the  heart,  and  lowering  the  blood 
pressure,  either  by  means  of  mild  counter-irritation  and  local 
depletion,  purgation,  venesection,  or  ligature  of  the  internal 
carotid  artery. 

Compression  may  be  relieved  by  lumbar  puncture,  tre- 
phining, or  lowering  the  blood  pressure.  Lumbar  puncture 
and  trephining  are  attended  by  certain  risks — namely,  a 
possible   tendency    to    bring    about    rupture    or  hemorrhage 


DISEASES  OF  THE  MIA  IN  203 

into  the  ventricles,  or  through  the  convexity  of  the  brain,  hy 
assisting  its  passage  along  the  lines  of  least  resistance. 

Best.  Complete  rest  in  bed  is  essential — the  patient  being 
laid  flat,  with  the  head  and  shoulders  slightly  raised,  Every- 
thing likely  to  constrict  the  neck  should  he  removed.  If 
the  patient  is  conscious,  no  muscular  effort  should  be  allowed, 
nor  under  any  circumstances  should  he  be  moved  more  than 
is  necessary. 

Active  treatment.  The  physician  should  be  guided  by  the 
state  of  the  blood  pressure  l  and  the  degree  and  severity  of 
the  symptoms  of  compression,  as  to  what  he  should  do  in 
a  case  of  cerebral  hemorrhage. 

(a)  If  the  arterial  pressure  is  not  high,  but  the  symptoms 
of  compression  severe,  local  depletion — e.g.,  the  application  of 
mustard  to  the  feet  or  abdomen,  or  mild  purgation — is 
sufficient.  A  moderate  quantity  of  cerebro-spinal  fluid  may 
also  be  withdrawn  by  lumbar  puncture. 

(b)  If  the  arterial  pressure  is  high  without  much  com- 
pression, a  sharp  purge  or  venesection  is  indicated. 

(c)  If  the  arterial  pressure  is  high  and  cerebral  com- 
pression profound,  the  blood  pressure  should  be  lowered  by 
a  sharp  purge  or  venesection,  and  the  pressure  symptoms 
relieved  by  lumbar  puncture  or  trephining — this  latter  being 
especially  useful  when  the  hemorrhage  is  subtentorial  in 
position  (pons  or  cerebellum).  If  the  hemorrhage  is  in  the 
cerebrum,  ligature  of  the  internal  carotid  artery  on  the  side 
of  the  lesion  is  preferable,  as  by  this  means  the  hemorrhage 
will  cease  and  further  compression  be  prevented,  while  the 
general  arterial  pressure  (including  that  of  the  basilar  artery) 
will  not  be  lowered,  nor  the  blood  supply  of  the  bulbar 
centres  diminished. 

In  ingravescent  hemorrhage,  purgation  or  venesection  are 
indicated.  If  compression  symptoms  remain  profound  after 
the  hemorrhage  has  ceased,  trephining  and  removal  of  the 
clot  may  be  carried  out. 

In  meningeal  hemorrhage,  trephining,  removal  of  the  clot, 
and  ligature  of  the  ruptured  artery  are  the  only  methods  of 
treatment. 

1  An  arterial  pressure  of  over  180  mm.  of  mercury  mar  be  regarded  as 
high  blood  pressure  ;  but  it  should  be  remembered  that  any  sudden  increase  of 
intracranial  pressure  causes  a  temporary  rise  of  the  blood  pressure. 


204 


NERVOUS   DISEASES 


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206  XKKYors  niSKASKS 


EEMIPLEGIA 


Hemiplegia,  or  paralysis  of  one  side  of  the  body,  is  the 
result  of  an  apoplectic  seizure,  whatever  be  the  nature  of  the 
underlying  lesion. 

For  descriptive  purposes  it  may  be  divided  into  two 
periods  :  (1)  that  observed  during  the  first  three  or  four  weeks 
following  '  the  stroke,'  when  it  is  characterised  by  more  or  less 
complete  unilateral  paralysis  with  liaccidity  or  only  incipient 
rigidity  of  the  limbs  ;  and  (2)  that  which  succeeds  the  above, 
and  determines  the  condition  of  chronic,  or  persistent,  hemi- 
plegic  weakness,  whose  features  are  partial  paralysis,  spasticity 
and  contracture  of  the  limbs,  and  sometimes  associated 
posthemiplegic  disorders  of  movement. 

1.  In  favourable  cases  the  hemiplegic  weakness  may 
rapidly  subside,  so  that  in  two  or  three  months  little  evidence 
of  the  previous  paralysis  may  be  observed,  although  defects  in 
the  finer  movements  of  the  hand  may  persist  for  an  indefinite 
period.  Even  in  cases  of  considerable  severity,  some  return 
of  movement  makes  its  appearance  within  three  or  four 
weeks,  so  that  the  patient  xa^y  be  able  to  rise  from  bed  and 
walk  with  assistance. 

The  method  of  recovery  of  the  paralysed  side  usually 
follows  certain  definite  lines.  The  common  observation  is 
that  the  face  recovers  before  the  leg,  and  the  leg  before  the 
arm.  The  coarser  movements  at  the  larger  joints  recover 
before  the  finer  movements  at  the  smaller  joints,  but  the 
more  specialised  movements  of  the  hand,  fingers,  and  thumb 
rarely  regain  the  previous  power  of  precise  movement. 

A  detailed  examination  of  the  motor  functions,  made 
during  the  first  month  after  the  stroke,  will  show  that  the 
lower  portion  of  the  face  is  considerably  more  paralysed  than 
the  upper  part.  The  tongue  is  protruded  towards  the  para- 
lysed side.  Some  weakness  of  the  movements  of  the  head 
and  neck  may  be  observed  when  movements  are  made  against 
resistance  to  the  paralysed  side.  The  arm  may  be  raised  a 
little  at  the  shoulder  joint,  but  the  movement  is  accompanied 
usually  by  considerable  pain  ;  flexion  and  extension  at  the 
elbow  are  feeble  ;  the  grasp,  if  present  at  all,  is  very  feeble. 
The  leg  may  be  drawn  up  at  the  hip  and  stretched  out  at  the 
knee,  but  dorsiflexion  of  the  ankle  is  feeble  if  performed  at 


DISEASES  OF  THE  BRAIN  207 

all.     The  trunk  movements  are  weakened  on  the  paralysed 
side. 

On  the  hemiplegic  side  the  tendon  jerks  are  exaggerated, 
an  ankle  clonus  and  extension  of  the  big  toe  are  usually 
present,  and  the  abdominal  reflexes  are  abolished,  or  impaired. 

Even  during  this  stage  some  rigidity  of  the  muscles  may 
be  detected  with  a  tendency  to  contracture  of  the  flexor  groups, 
so  that  passively  straightening  the  fingers,  wrist,  or  elbow  may 
be  accompanied  by  resistance.  The  mechanical  irritability  of 
the  muscles  is  increased. 

The  distribution  of  the  paralyses  and  the  presence  of 
associated  hemiansesthesia,  hemianopsia,  and  aphasia  depend 
upon  the  position  of  the  lesion. 

2.  Although  the  clinical  course  of  a  hemiplegia  has  been 
divided  into  two  stages,  there  is  no  hard-and-fast  line  of 
demarcation,  the  late  emerging  gradually  out  of  the  early. 
The  most  prominent  of  the  symptoms  of  the  later  stages 
are  spasticity  and  contracture  of  the  limbs,  in  which  flexion 
predominates  over  extension,  and  pronation  over  supination. 
Hence  the  arm  is  adducted  at  the  shoulder  and  flexed  at  the 
elbow,  the  forearm  is  in  a  state  of  pronation,  the  fingers 
are  clenched,  and  the  thumb  flexed  and  opposed  into  the 
palm.  The  thigh  is  adducted  at  the  hip  joint,  the  knee  is 
rigid,  the  heel  slightly  drawn  up,  the  foot  inverted,  and  the 
toes  pointed.  The  attitude  and  gait  of  a  hemiplegic  person 
are  in  consequence  highly  characteristic.  The  paralysed  side 
seems  to  move  as  a  whole,  and  to  be  lower  than  the  normal 
side.  The  arm  is  carried  in  the  position  just  described  ;  the 
leg  is  moved  in  a  rigid  fashion,  the  foot  being  carried  forward 
in  the  arc  of  a  circle,  the  toes  and  inner  edge  of  the  foot 
scraping  the  floor. 

These  patients  are  said  when  turning  to  pivot  upon  the 
non-paralysed  side,  but  there  does  not  seem  to  be  any 
constancy  in  this  phenomenon. 

The  mental  condition  also  shows  prominent  changes  in 
many  cases.  Alterations  in  temperament  and  disposition  are 
stated  to  have  occurred  since  the  stroke.  The  memory  is 
often  impaired,  and  a  marked  tendency  to  loss  of  emotional 
control  is  obvious — these  patients  laughing,  but  more  usually 
crying,  on  the  slightest  provocation.  They  frequently  become 
apathetic  and  indifferent  to  their  surroundings,  and  a  previous 


208  NERVOUS  DISEASES 

natural  tendency  to  effort  and  activity  may  be  replaced 
by  timidity  and  want  of  decision.  It  is  difficult,  however, 
to  say  how  far  the  posthemiplegic  dementia  may  be 
attributed  directly  to  the  focal  lesion  or  to  the  accompanying 
arterio-sclerosis. 

Treatment.  Much  may  be  done  in  the  weeks  succeeding 
the  stroke  to  prevent  or  retard  the  onset  of  the  rigidities, 
contractures,  and  arthritic  changes  which  characterise  so 
many  chronic  hemiplegics.  In  order  to  be  of  much  avail, 
massage  and  passive  and  active  movements  against  resistance 
require  to  be  commenced  early  and  continued  for  a  long  time, 
usually  for  several  months. 

Treatment  should  therefore  be  commenced  as  soon  as  the 
immediate  effects  of  the  stroke  have  passed  away.  Of  most 
service  are  massage  and  passive  movements  ;  but  the  faradic 
currents  may  be  of  use  when  rigidity  is  slight  or  absent.  The 
patient  should  also  be  encouraged  to  use  his  limbs  as  freely 
as  possible :  daily  practice  with  some  form  of  '  exerciser ' 
being  of  distinct  advantage.  For  the  arthritic  pain, 
radiant-heat  baths,  combined  with  passive  movements,  are 
useful. 

INTRACRANIAL   ANEURISM 

The  description  given  here  refers  only  to  aneurism  of  the 
larger  cerebral  arteries. 

Etiology.  Aneurism  is  more  frequent  in  males  than  in 
females.  It  occurs  especially  from  the  age  of  ten  years 
onwards  to  sixty,  but  is  more  common  in  mid-adult  life  than 
at  either  extreme.  The  most  common  cause  is  embolism  of  a 
cerebral  artery — occurring  in  association  with  endocarditis, 
syphilitic  disease  of  the  arteries,  and,  in  rare  instances, 
arterial  degeneration  and  traumatism. 

Frequency.  The  internal  carotid  arterial  system  is  more 
often  the  seat  of  aneurism  than  the  vertebral.  The  arteries 
affected  are  in  order  of  frequency  (according  to  Gowers),  the 
middle  cerebral,  basilar,  internal  carotid,  anterior  cerebral, 
posterior  communicating,  anterior  communicating,  vertebral, 
posterior  cerebral,  and  inferior  cerebellar.  Aneurisms  affecting 
the  vessels  of  the  basal  ganglia  and  centrum  ovale  are  rare, 
except  in  the  miliary  form. 


DISEASES   OF  THE   BEAIN  209 

Symptoms.  The  symptoms  produced  by  aneurism  depend 
more  upon  the  situation  than  upon  the  size  of  the 
swelling. 

In  the  embolic  variety  rupture  is  liable  to  occur  early,  on 
account  of  the  softened  condition  of  the  vessel  walls,  and  is 
often  the  first  indication  of  the  morbid  condition.  The 
arterial  dilatation  which  occurs  in  these  cases,  even  when 
pressing  upon  nervous  structures,  does  not  give  rise  to 
pressure  symptoms. 

In  cases  of  slowly  growing  aneurism,  with  firm  walls, 
symptoms  of  compression  or  destruction  of  the  surrounding 
tissues  may  appear  early  in  the  course  of  the  disease. 

From  the  standpoint  of  symptoms,  aneurisms  may  be 
divided  into  :  (a)  those  in  which  no  symptoms  were  observed 
during  life  (18  per  cent.) ;  (b)  those  in  which  there  were 
indications  of  cerebral  tumour  (16  per  cent.)  ;  (c)  those  in 
which  symptoms  of  tumour,  or  other  cerebral  lesion,  were 
followed  by  fatal  apoplexy  (20  per  cent.)  ;  and  (d)  those  in 
which  the  first  indication  was  an  apoplectic  seizure  (46  per 
cent.).1 

Out  of  555  cases  of  cerebral  aneurism,  372  died  of 
apoplexy.  In  339  of  these  the  apoplectic  stroke  was  due 
to  rupture  of  the  aneurism ;  while  in  the  remainder,  the 
hemorrhage  was  due  to  the  rupture  of  a  blood-vessel  not 
affected  by  aneurism.1 

General  symptoms  are  frequently  absent,  but,  when  present, 
pain  in  the  head  is  usually  associated  with  buzzing  noises. 
Optic  neuritis  is  rare,  but  is  probably  due  to  the  same  causes 
as  in  intracranial  tumour.  Noises  in  the  head — such  as 
singing  and  buzzing — do  not  occur  out  of  proportion  to  their 
frequency  in  general  intracranial  diseases. 

The  hearing  of  a  murmur  on  auscultation  of  the  skull, 
which  has  been  referred  to  in  this  connexion  by  many  writers, 
is  of  no  value,  as  it  has  only  been  verified  in  two  out  of  555 
cases  (Beadles).  It  has  also  been  heard  in  cases  in  which  no 
aneurism  was  present,  as  in  hydrocephalus,  Graves's  disease, 
and  anaemia.  A  murmur  has  also  been  heard  in  cases  of 
vascular  tumour  of  the  brain,  and  when  a  tumour  has 
pressed  upon  a  cerebral  artery. 

1  Beadles,  Brain,  1907. 

14 


210  NERVOUS  DISEASES 

The  mental  dullness  and  irritability  which  have  been 
stated  to  be  symptoms  of  cerebral  aneurism  are  in  reality 
due  to  general  arterio- sclerotic  changes. 

The  only  sign  of  any  real  significance  is  the  intermittent 
character  of  the  symptoms. 

Focal  symptoms.  Aneurism  of  the  cavernous  part  of  the 
internal  carotid  artery  is  shown  by  paralysis  of  the  ocular 
muscles,  the  third  nerve  being  first  affected,  in  association 
with  homolateral  blindness  from  pressure  upon  the  optic  nerve. 

In  the  case  of  the  anterior  cerebral  artery,  loss  of  sight 
and  of  smell  may  be  present  from  pressure  upon  the  optic 
and  olfactory  nerves. 

Third  nerve  paralysis  and  homonymous  hemianopsia  to 
the  opposite  side  may  be  found  in  aneurism  of  the  posterior 
communicating   artery. 

In  the  case  of  the  middle  cerebral  artery,  convulsions 
followed  by  paralysis  of  a  focal  character  simulating  cerebral 
tumour  have  been  described. 

Aneurism  of  the  basilar  artery,  in  its  anterior  part,  has 
been  associated  with  hemiplegia  from  pressure  upon  the  crus 
cerebri ;  in  its  posterior  part,  with  symptoms  of  pressure  upon 
the  pons.  According  to  its  lateral  and  antero-posterior 
position,  pressure  symptoms  are  referred  from  the  fifth 
cranial  nerve  downwards.  In  a  few  cases  of  basilar  aneurism, 
movements  of  the  head  have  given  rise  to  temporary 
respiratory  difficulty  from  pressure  upon  the  medulla. 

In  aneurism  of  the  posterior  cerebral  artery,  palsy  of  the 
third  and  sixth  nerves  with  a  crossed  hemiplegia  has  been 
described. 

The  cerebellar  arteries  are  rarely  affected. 

The  diagnosis  of  cerebral  aneurism  is  practically  impos- 
sible ;  but  in  a  case  with  focal  symptoms  referred  to  the 
position  of  one  of  the  cerebral  arteries  as  above  described,  in 
which  the  symptoms  are  intermittent  in  character,  without  the 
accompanying  general  symptoms  of  intracranial  tumour,  and 
in  which  either  a  history  of  syphilis  or  of  endocarditis  is 
obtained,  the  presumption  is  in  favour  of  cerebral  aneurism. 
The  X-rays  might  prove  of  assistance. 

The  treatment  consists  of  rest  in  bed,  with  large  doses  of 
potassium  iodide  internally.  If  the  aneurism  can  be  localised 
with  certainty,  the  internal  carotid  artery  should  be  ligatured. 


DISEASES  OF  THE  BRAIN  211 


[iliary  Aneurisms 


These  are  minute  dilatations  of  the  cerebral  arterioles. 
They  are  extremely  small,  and  very  numerous.  They  are 
rarely  found  under  forty  years  of  age.  They  are  most 
frequent  in  the  vessels  supplying  the  basal  ganglia,  then 
the  cortex  cerebri,  pons,  cerebellum,  and  centrum  ovale. 

A  weakening  of  the  muscular  coat,  so  that  the  vessel  wall 
consists  only  of  the  intimal  and  adventitial  sheaths,  is  the 
pathological  change  giving  rise  to  these  arterial  dilatations. 
Owing  to  this  change,  the  vessel  wall  yields  to  the  intra- 
vascular pressure. 

Their  rupture  is  a  common  cause  of  cerebral  hemorrhage. 


Chapter  IV 

THE  CEREBRAL  PARALYSES  OF  INFANCY  AND 
CHILDHOOD 

The  various  forms  of  cerebral  paralyses  occurring  in  infants 
and  children  present  many  features,  which  are  quite  distinct 
from  those  seen  in  adults.  This  is  accounted  for  by  the 
undeveloped  state  of  the  infant  brain,  by  the  pathological 
processes  which  underly  their  production,  and  by  the  different 
effects  which  similar  lesions  produce  in  the  infant  and  the 
adult  brain.  The  paralysis  may  be  monoplegic,  hemiplegic, 
paraplegic,  and  diplegic.  The  symptoms  may  be  progressive 
and  regressive.  The  mental  condition  may  be  unimpaired 
or  show  any  degree  of  enfeeblement  to  complete  idiocy  or 
amentia. 

The  lesions  which  give  rise  to  cerebral  paralyses  in  children 
are  the  following : — 

Vascular  lesions.  (1)  Thrombosis.  This  may  be  arterial, 
venous,  or  combined  arterial  and  venous.  It  is,  as  a  rule, 
associated  with  marasmus,  or  an  enfeebled  state  of  the 
circulation  following  an  acute  illness. 

If  the  arrest  of  the  blood  supply  occurs  during  foetal  or 
early  infant  life,  the  pathological  picture  is  distinct  from  that 
seen  in  later  life.  If  the  arrest  is  incomplete,  either  atrophic 
sclerosis,  or  softening  with  secondary  cystic  formation,  results. 

14* 


212  NERVOUS  DISEASES 

If  complete,  certain  portions  of  the  brain  may  disappear, 
leaving  well-defined  cavities,  as  if  portions  of  the  brain  had 
been  punched  out  with  a  cheese-scoop.  This  condition  is 
known  as  acquired  porencephaly,  and  may  only  be  discovered 
at  autopsy. 

Thrombosis  occurring  in  later  childhood  resembles,  both 
in  its  pathological  and  clinical  characters,  that  which  takes 
place  in  adults. 

2.  Hemorrhage.  Cerebral  hemorrhage  is  not  common 
in  infancy  or  childhood.  It  may  occur  as  the  result  of 
trauma,  of  rupture  of  a  vessel  into  a  softened  area,  in  some 
blood  diseases,  and  in  the  form  of  capillary  hemorrhages  in 
whooping  cough. 

Meningeal  hemorrhage  is  not  uncommon  from  injury  at 
birth,  when  the  brain  suffers  not  so  much  from  internal 
injury  as  from  pressure.  This  form  of  hemorrhage  occurs 
chiefly  from  the  small  veins  running  into  the  superior 
longitudinal  sinus. 

3.  Embolism.  This  is  rare  in  foetal  life,  and  only  occurs 
in  young  children  as  the  result  of  endocarditis  or  general 
blood  infection. 

Encephalitis,  or  non-purulent  inflammation  of  the  brain. 
Encephalitis  is  probably  the  commonest  cause  of  cerebral 
paralysis  in  infancy.  The  nature  of  the  lesion  is  identical 
with  that  seen  in  acute  poliomyelitis,  and  may  result  in 
almost  complete  recovery,  or  leave  permanent  damage  in 
the  shape  of  cicatrices  or  cystic  cavities.  Not  a  few  cases 
terminate  fatally  during  the  acute  stage. 

Traumatic  encephalitis  may  be  caused  by  damage  to  the 
skull  or  brain,  and  may  be  complicated  by  meningeal  or 
intracerebral  hemorrhage. 

Agenesia.  As  the  several  systems  of  the  brain  develop  at 
different  ages,  a  lesion  may  cause  either  destruction  of  any 
particular  system  or  arrest  of  its  development.  There  would 
also  appear  to  be  in  some  cases  a  congenital  tendency  to  arrest 
of  development  or  early  death  of  the  neurone  (abiotrophy). 

CONGENITAL  CEREBRAL  DIPLEGIA 

This  condition  arises  from  an  inherited  taint,  which  may 
originate  either  during  intrauterine  life  or  after  birth.     The 


DISEASES  OF  THE  BRAIN  213 

influences  at  work  are  either  poisons  introduced  through  the 
mother  during  intrauterine  life,  or  directly  inherited  from 
one  or  other  parent.  Owing  to  these  causes  the  nervous 
structures  may  never  develop,  may  not  attain  maturity,  or 
may  degenerate  after  development.  Clinical  examples  of  these 
are  seen  in  children  who  never  learn  to  walk,  who  walk  late 
but  never  properly,  or  who,  having  learned  to  walk,  gradually 
become  unable  to  do  so. 

Not  infrequently  one  physiological  system  of  neurones  is 
alone  affected,  but  commonly  two  or  more  may  suffer.  It  is 
therefore  obvious  that  in  cases  of  diplegia  the  clinical  con- 
dition varies  according  to  the  neuronic  systems  involved.  In 
some  cases  the  disability  is  almost  exclusively  motor,  in  others 
visual  or  sensory,  and  in  others  psychical.  The  disease  is 
rarely  restricted  to  one  system,  and  most  cases  exhibit  a 
proneness  to  convulsions  and  mental  deterioration. 

Morbid  anatomy.  The  convolutions  are  firm  and  atro- 
phied, and  the  sulci  wide  ;  but  the  general  shape  of  the  cortex 
is  preserved  according  to  the  state  of  development  at  which 
the  regressive  changes  commenced.  If  convolutionary  develop- 
ment has  taken  place,  the  appearances  observed  are  known  as 
the  'walnut  brain.'  Here  the  neuroglial  tissue  is  proliferated, 
and  the  nerve  elements  are  absent  or  degenerated.  There  is 
no  evidence  of  vascular  or  inflammatory  changes.  Primary 
atrophy  or  degeneration  of  one  neuronic  system  is  combined 
with  a  secondary  atrophy  of  those  in  physiological  association 
with  it. 

Symptoms.  In  most  cases  the  disease  is  evident  at  or 
shortly  after  birth.  In  other  cases  the  symptoms  may  not 
show  themselves  until  the  child  reaches  an  age  at  which 
it  ought  to  walk  and  talk.  In  familial  cases  the  onset  is 
frequently  delayed  until  after  the  sixth  year.  The  sexes 
suffer  equally. 

The  chief  clinical  characteristic  is  rigidity  or  spasticity, 
associated  with  paresis  or  paralysis.  This  is  bilateral,  though 
sometimes  one  side  suffers  more  than  the  other.  The  whole 
body  may  be  affected,  the  face  relatively  less  than  the  limbs, 
but  in  other  cases  the  spastic  weakness  is  limited  to  the 
lower  limbs  (Little's  disease).  Deformities  due  to  spastic 
contracture  are  frequent.     (Fig.  62.) 

The  mental  deficiency  varies  from  mere  backwardness   to 


I'll 


NERVOUS  DISK  ASKS 


absolute  and  complete  idiocy.  h\  the  slight  cases  the  mental 
condition  is  characterised  by  backwardness,  slowness  in 
talking,  and  impaired  intelligence.  More  unfavourable  are 
excitability,  restlessness,  destructive  tendencies,  and  dirty 
habits. 

The   mental    condition  bears   no  relation   to  the  physical 
infirmities. 

Cranial    nerves.      Bilateral   primary   optic   atrophy   from 

degeneration  of  the  gan- 
glion cells  of  the  retina 
is  seen  in  a  considerable 
number  of  cases. 

Inequality  of  the  pupils 
with  feeble  reaction  to  light 
is  not  uncommon.  Hippus 
is  frequently  seen.  Isolated 
palsies  of  the  third  and 
sixth  nerves  may  be  pre- 
sent ;  but  impairment  of 
ocular  movements  —  such 
as  spontaneous  nystagmus 
or  strabismus — are  more 
frequent.  Convergent 
squint  is  common,  but 
divergent  is  rare. 

Spastic  weakness  of  the 
facial  muscles  may  or  may 
not  be  present.  It  is  some- 
times accompanied  by  a 
similar  condition  of  the 
tongue  and  palate.  The 
child  may  be  unable  to  protrude  the  tongue,  which  is 
often  large,  articulation  is  impaired,  and  saliva  may 
dribble  from  the  corners  of  the  mouth.  Swallowing  is 
slowly  performed— a  long  time  being  taken  over  the  bottle. 
If  made  to  cry,  great  facial  overaction  occurs  with  little 
or  no  proportionate  sound. 

Motor  system.  Rigidity  may  be  generalised,  or  limited  to 
the  lower  limbs.  When  extreme,  it  is  possible  to  lift  the 
child  up  in  rigid  extension.  In  less  severe  degrees,  ^  the 
child  is  unable  to  sit  owing  to  inability  to  flex  the  thighs. 


Pig.62.  Photograph  of  a  case  of  cerebral 
diplegia,  showing  paralysis  and  spas- 
ticity witli  a  tendency  to  contractures. 
The'left  limbs  are  more  affected  than 
the  right.     The  face  is  also  involved. 


DISEASES  OF  THE  BRAIN 


215 


When  walking  is  possible,  the  gait  may  be  that  known  as 
'  scissor  gait,'  or  cross-legged  progression,  the  feet  being 
pointed  and  inverted,  the  knees  slightly  flexed,  and  the  thighs 
strongly  adducted  and  rotated  inwards.  Milder  degrees  of 
spastic  paraplegia  are  also  found.  In  some  cases  the  patient 
may  not  be  able  to  bring  his  heels  to  the  ground,  but  walks 
on  the  balls  of  the 
toes  (digitigrade  gait). 
(Fig.  63.) 

Paresis  exists  in  all 
cases  where  rigidity  is 
marked,  but  rigidity 
may  be  present  with 
out  much  motor  weak- 
ness. 

In  severe  cases 
characteristic  attitudes 
may  be  assumed.  The 
head  is  bent  forward 
and  the  spinal  column 
may  show  either  a 
kyphotic  curve  or 
lordosis.  The  upper 
limbs  may  or  may  not 
be  affected,  but  are 
never  involved  to  the 
same  extent  as  the 
lower.  The  upper  arm 
is  adducted,  the  fore- 
arm flexed  and  supi- 
nated,  and  the  hand 
flexed  at  the  wrist. 
The  lower  limbs  are  extended  and  adducted  at  the  hip, 
flexed  at  the  knee,  and  the  feet  are  in  the  position  of  pes 
cavus  or  talipes  equino-varus.  The  muscles  may  be  firm  or 
hard  to  the  touch,  and  in  cases  of  athetosis  hypertrophy 
may  occur.  In  the  latest  stages  wasting  and  fibrillary 
contraction  with  impaired  faradic  and  myotatic  irritability 
are  observed. 

The  sensory  system  may  not  be  affected. 

The  reflexes  are  characteristic   of   spastic  paralysis.     The 


Fig.  63. — Shows  the  cross-legged  or  *  scissor 
gait '  of  congenital  cerebral  diplegia. 


216  NERVOUS  DISEASES 

deep  are  increased,  the  superficial  often  unaffected,  and  the 
plantars  are  extensor  in  type. 

The  sphincters.  Incontinence  is  common,  but  is  mainly 
due  to  the  mental  deficiency.     Precipitancy  may  occur. 

Disorder*  of  movement.  All  degrees  of  disordered  move- 
ment are  present — from  clumsiness  and  overaction  of  a  slow 
spastic  type  to  well  marked  intention  tremor  like  that  seen 
in  disseminated  sclerosis.  Very  characteristic  are  bilateral 
athetoid  and  choreiform  movements,  which  may  be  found  in 
cases  in  which  rigidity  is  present  with  little  motor  weakness 
(athetotic  diplegia). 

Speech  defects.  In  the  congenital  cases  speech  may  never 
be  acquired.  In  those  cases  which  commence  after  the  child 
has  learned  to  speak,  motor  aphasia — sometimes  temporary 
at  other  times  partial  and  complete — maj^  be  observed. 

In  nearly  all  cases  speech  is  slowly  acquired.  Articulation 
is  slow,  hesitating,  or  stammering,  and  accompanied  by  facial 
overaction  and  slobbering.  It  not  infrequently  assumes  a 
form  in  which  the  syllables  are  pronounced  in  an  unusual 
fashion — such  as  lalling. 

Although  unable  to  speak,  these  children  appear  in  some 
cases  to  understand  what  is  said  to  them. 

Convulsions  are  commonly  associated  with  cerebral  diplegia, 
epilepsy  occurring  in  about  10  per  cent,  of  the  cases. 

The  following  types  of  cerebral  diplegia  are  found  : — 

1.  Generalised  rigidity  with  bilateral  paralysis,  and  more 
or  less  mental  impairment. 

2.  Imbecility  or  idiocy,  with  slight  or  no  motor  weakness. 

3.  Paraplegia— Little's  disease. 


ACQUIRED   CEREBRAL  PARALYSES 
Infantile  Hemiplegia  and   Diplegia 

Acquired   infantile    hemiplegia    and    diplegia   are    of    the 
following  kinds  : — 

1.  Birth  palsies.    These  arise  from  meningeal  hemorrhage 
and  trauma. 

2.  Acquired  palsies.     These  are  due  to  : 

(a)  Encephalitis— primary,  traumatic  following  acute 
infective  diseases,  and  suppurative. 


DISEASES  OF  THE  BRAIN  217 

(b)  Vascular  lesions — thrombosis  (arterial  and  venous;, 
hemorrhage  (meningeal  and  cerebral),  and 
embolism. 

Encephalitis  is  the  pathological  lesion  in  the  majority  of 
cases  of  infantile  hemiplegia.  Although  frequently  occurring 
as  a  sporadic  disorder,  it  is  occasionally  met  with  in  epidemic 
form,  and  is  especially  common  during  the  seasonal  period 
in  which  poliomyelitis  also  occurs   (p.  438). 

Morbid  anatomy.  Encephalitis  is  an  acute  inflamma- 
tory process  in  and  around  the  blood-vessels  of  the  brain, 
characterised  by  hyperemia,  hemorrhagic  exudation,  cellular 
infiltration,  and  multiple  small  hemorrhages.  It  may  have  a 
local  or  a  general  distribution,  and  affect  some  regions  more 
than  others.  The  grey  matter,  owing  to  its  greater  vascu- 
larity, suffers  more  than  the  white,  the  changes  being  best 
seen  in  the  cortex  and  basal  ganglia.  Similar  changes  also 
occur  in  the  central  grey  matter,  floor  of  the  fourth  ventricle, 
and  the  anterior  horns  of  the  spinal  cord.  The  terms 
encephalitis  and  poliomyelitis  (superior,  inferior,  and  an- 
terior), are  based,  therefore,  on  anatomical  grounds. 

When  the  cerebral  cortex  is  affected,  the  pia  mater  shares 
in  the  inflammation.  The  brain  substance  is  red,  swollen, 
and  softened,  and  the  distinction  between  grey  and  white 
matter  is  obliterated.  Numerous  small  hemorrhages  are 
also  present.  The  cerebro-spinal  fluid  is  increased  in  the 
ventricles. 

Microscopically,  the  small  blood-vessels  are  found  distended 
and  ruptured.  A  perivascular  cell  proliferation  is  also  found, 
and  the  vessel  walls  may  show  fatty  degeneration.  In  severe 
cases  small  foci  of  degenerated  myeline  and  fatty  and  granular 
debris  are  also  seen.  The  nerve  cells  may  be  either  destroyed, 
show  fatty  and  hyaline  degeneration,  or  only  chromatolysis,the 
observed  changes  depending  upon  the  intensity  of  the  inflam- 
mation ;  even  in  severe  cases  many  nerve  cells  escape.  Either 
resolution  or  destruction  of  portions  of  the  brain  results.  If 
the  latter,  the  changes  consist  of  an  increase  of  the  neuroglia 
with  the  formation  of  scars  or  sclerotic  patches.  Secondary 
degeneration  of  nerve  fibres  is  also  found. 

Its  onset  is  most  common  during  the  first  three  years 
of  life,  contrasting  in  this  way  with  thrombosis,  which  is  more 
common  about  the  fifth  or  sixth  year. 


218  NERVOUS  DISEASES 

The  sexes  are  about  equally  affected.  Parental  alcoholism 
and  syphilis  may  be  predisposing  influences. 

Symptoms.  The  onset  of  the  disease  is  sudden,  with 
convulsions,  fever,  vomiting,  and  sometimes  coma.  After  a 
few  days  these  general  symptoms  begin  to  subside,  and  it 
is  then  noticed,  if  not  before,  that  the  child  is  paralysed. 
The  paralysis  is  first  of  the  flaccid  type,  but  within  a  week 
signs  of  spasticity  develop.  In  mild  cases  the  paralysis 
may  be  slight  and  transitory  ;  in  severe  cases,  on  the  other 
hand,  spasticity  and  contracture  set  in  early,  and  may  be 
associated  with  trophic  disturbance  and  arrest  or  impairment 
of  growth  on  the  paralysed  side. 

The  paralysis  at  first  has  a  more  or  less  general  distribu- 
tion. If  encephalitis  is  widespread  and  severe,  permanent 
bilateral  pals}'  (diplegia)  may  result.  In  many  cases  the 
palsy  is  confined  to  one  side  (hemiplegia) .  Occasionally  it  is 
more  or  less  limited  to  one  limb,  but  even  in  such  cases  reflex 
changes  indicative  of  a  unilateral  lesion  are  present.  In  other 
cases  the  sensory  and  psychical  regions  of  the  brain  may  be 
chiefly  implicated,  when  sensory  symptoms  and  mental 
changes  of  a  transitory  or  permanent  nature  may  to  a  large 
extent  replace  the  paralytic  phenomena. 

The  basal  ganglia  and  central  grey  matter  of  the  mid- 
brain may  be  involved  conjointly  with  the  cortical  tissues,  or, 
as  a  primary  condition,  characterised  by  permanent  oculo- 
motor paralysis  and  disorders  of  movement. 

In  infantile  hemiplegia,  the  face  is  less  affected  than  the 
arm  or  leg.  The  facial  paralysis  gives  rise  to  some  stiffness 
and  want  of  expression.  The  emotional  movements  are  more 
impaired  than  the  voluntary.  Not  infrequently  slow  mobile 
spasm  or  choreiform  movements  develop  at  a  later  period. 

Cranial  nerves.  Strabismus  and  ocular  palsies  sometimes 
occur  at  the  onset  of  hemiplegia,  in  which  respect  the  infantile 
differs  from  the  adult  form.     (Fig.  64.) 

Hemianopsia  may  be  found  in  some  cases,  especially  in 
association  with  hemianesthesia. 

Motor  system.  The  upper  limb  shows  spasticity  and 
contracture ;  and  trophic  disturbances,  causing  diminished 
growth  with  blueness  and  coldness  of  the  limb,  further 
characterise  the  condition.  The  arm  is  adducted  at  the 
shoulder,    the   elbow   flexed   at   a   right   angle,    the  forearm 


DISEASES  OF  THE  BRAIN 


219 


pronated,  the  wrist  flexed  and  deviated  to  the  ulnar  side,  and 
the  thumb  and  fingers  firmly  flexed.  Paralysis  of  the  distal 
parts  of  the  limb  is  more  pronounced  than  the  proximal. 

The  lower  limb  presents  the  same  general  features,  though 
in  less  degree.  When  the  palsy  has  been  severe,  the  lower 
limb  may  be  shortened  from  trophic  changes ;  but  even 
without  these  the  contractures  may  give  rise  to  shortening. 
The  thigh  is  adducted  and  rotated 
inwards,  the  knee  slightly  flexed, 
the  heel  drawn  up,  the  foot 
inverted,  and  the  toes  flexed.  In 
other  cases  pes  cavus,  or  talipes 
equinus  or  equino-varus,  is  pre- 
sent.    (Fig.  64.) 

The  gait  resembles,  generally, 
that  seen  in  adults.  Even  in  cases 
where  the  leg  is  severely  para- 
lysed, the  disability  as  regards 
walking  is  more  apparent  than 
real.  The  leg  is  circumducted  at 
the  hip,  and  may  be  carried 
across  the  middle  line  at  the  end 
of  the  movement.  The  patient 
walks  upon  the  toes,  and  if  much 
shortening  exists,  a  compensatory 
tilting  of  the  pelvis  results. 

Sensation  may  not  be  impaired, 
but  in  other  cases  hemi-anaesthesia    Fig.  64.^Shows  the  appearance 
may  be  present. 

The  reflexes.    The  tendon  jerks 
are  characteristic    of    hemiplegia. 
The  superficial  reflexes  may  be  normal.      The  plantar s    are 
extensor. 

The  sphincters  are  unaffected. 

Trophic  changes  are  seen  in  arrested  or  impaired  growth 
of  the  limbs,  vaso-motor  disturbances,  contractures,  and  in 
some  cases  malformation  of  the  bones. 

Speech  defects.  If  the  child  has  not  learned  to  speak, 
the  acquisition  of  speech  may  be  slow ;  but,  as  a  rule,  it  is 
eventually  acquired.  If  the  lesion  occurs  when  the  child  is 
learning  to   speak,  and   involves   the    motor    speech  centre. 


and  attitiide  of  a  case  of 
infantile  hemiplegia.  There 
is  a  divergent  strabismus  of 
the  left  eve  in  this  case. 


220 


NERVOUS  DISEASES 


aphasia  results  ;  but  speech  is  slowly  regained  by  the  re-educa- 
tion of  the  other  side  of  the  brain.  Aphasia  may  be  a  purely 
transient  symptom,  in  cases  where  the  speech  centres  are 
slightly  involved.     If  the  speech  centres  are  involved,  various 

forms  of  motor  and 
sensory  aphasia  may 
result,  which  may  be 
permanent,  if  the 
mental  condition  is 
defective  or  the  child 
is  under  six  years  of 
a^e. 

Quite  apart  from 
aphasia  is  the  arti- 
culatory  disturbance 
and  over  -  action, 
depending  upon 
spasticity  and  inco-ordination  of  the  muscles  subserving 
articulation. 

Disorders  of  movements.  One  of  the  most  characteristic 
features  of  infantile  hemiplegia  is  the  development  of  various 
forms  of  irregular  movement  in  the  paralysed  limbs.     These 


i'h..  (So. 


A  view  of  the  palm,  of  tin-  hand  in  a 
case  of  ;ii  hetosis. 


Fig.  66. — Showing  athetotic  spasm  of  the  great  and  small  toes. 

disorders  may  either  occur  on  voluntary  effort  or  take  place 
spontaneously.     (Figs.  65  and  66.) 

(a)  Those  occurring  on  voluntary  effort.    Owing  to  stiffness 
or  rigidity  movements  are  carried  out  slowly  and  clumsily  with 


DISEASES  OF  THE  BRAIN 


221 


some  overaction.  Repeated  efforts  usually  diminish  the  stiff- 
ness, with  a  corresponding  improvement  in  the  movement. 
It  is  obvious  that  this  disorder  of  movement  can  only  occur 


Fig.  67. 


Fig.  68. 


Fig.  69. 


Fig.  70. 


Figs.  67-70. — Four  figures  showing  the  attitude  assumed  by  the  hauds 
and  fingers  in  a  case  of  athetosis. 


in  limbs  in  which  a  considerable  degree  of  voluntary  power 
exists.  Less  distinctive,  but  frequently  observed,  are  coarse 
or  fine  tremor,  inco-ordination,  and  rapid  jerky  movements. 

(6)   The  spontaneous  movements   are   chiefly  athetosis   or 
mobile  spasm.     This  develops  late,  and  ma}^  not  supervene 


222  XKKYOrS  DISEASES 

for  several  years  after  the  onset  of  the  paralysis.  Its  develop- 
ment signifies  that  no  further  recovery  from  the  paralysis  will 
take  place. 

Athetoid  movements  are  seen  in  the  distal  portion  of  the 
arm.  The  wrist  remains  more  or  less  flexed,  the  lingers  and 
thumb  slowly  disengage  themselves  and  become  strongly  hyper- 
extended,  spreading  out  in  a  fanlight  fashion.  The  ringers 
are  widely  abducted  and  wander  individually  or  conjointly 
with  a  semi-methodical  irregularity.     (Figs.  67  70.) 

Although  athetosis  is  involuntary,  it  is  occasionally  set  up 
on  attempts  at  voluntary  movements. 

Choreiform  disorders  occur  in  the  form  of  irregular  move- 
ments, and,  unlike  athetosis,  often  involve  the  proximal  parts 
of  the  limbs. 

Convulsive  seizures.  At  the  onset  of  the  disease,  generalised 
convulsions  are  invariably  present. 

Seventy-eight  per  cent,  of  the  cases  of  infantile  hemi- 
plegia become  epileptic.  The  seizures  may  either  directly 
supervene  on  the  original  convulsive  attack,  or  several  years 
may  elapse  between  the  onset  of  the  hemiplegia  and  the  first 
epileptic  seizure.  The  attacks  have  the  features  of  the 
epileptic  seizures  elsewhere  described,  the  convulsions  having 
a  tendency  to  chiefly  implicate  the  paralysed  limbs.  There 
is  frequently  a  local  aura,  beginning  in  the  paralysed  hand  or 
arm.  Other  auras  may  point  to  irritation  of  a  sensory  cortical 
area,  with  subsequent  generalised  convulsion.  Attacks  of 
minor  and  of  psychical  epilepsy  may  also  occur. 


PORENCEPHALY 

Two  varieties  of  this  form  of  cystic  formation  in  the 
cerebrum  are  observed :  one  developmental,  the  other  secon- 
dary, to  cutting  off  of  the  blood  supply — pseudo-  or  acquired 
porencephaly.  The  developmental  nature  of  the  first  variety 
is  proven  by  its  not  infrequent  association  with  other  similar 
conditions — such  as  cavity  formation  within  the  spinal  cord 
(syringomyelia)  and  spina  bifida.  The  cavity  is  more  or  less 
funnel-shaped,  and  always  communicates  with  the  ventricle  and 
sometimes  w7ith  the  subarachnoid  space. 

The   acquired   variety  may  be  found  in  any   part    of  the 


DISEASES  OF  THE   BRAIN 


22.'5 


brain,  but  is  more  common  over  the  Rolandic  and  parietal 
regions.  All  degrees  of  porencephaly  are  seen— from  a  mere 
dimple  on  the  surface  to  well-marked  cystic  formation. 

Clinically,  the  developmental  form  may  be  suspected 
when  other  signs  of  malformation  (meningocoele)  are  asso- 
ciated with  mental  deficiency  or  hemiplegia  weakness.  In 
the  acquired  form,  the  symptoms  depend  on  the  situation  of 
the  lesion.  The  existence  of  porencephaly  may  be  suspected; 
but  in  most  cases  it  remains  undetected,  until  demonstrated 
at  the  autopsy,  perhaps  many  years  afterwards. 


Fig.  71. — The  brain  of  a  young  adult  showing  atrophic  sclerosis  and 
acquired  porencephaly  as  described  in  the  text  (p.  223). 


ATROPHIC  SCLEROSIS  AND  CYSTIC  FORMATION 

In  Fig.  71  a  brain  is  depicted  showing  both  condi- 
tions in  the  same  hemisphere.  The  following  features  are 
observed :  first,  a  marked  diminution  in  the  size  of  the 
affected  hemisphere ;  and  secondly,  the  presence  of  an 
extensive   cyst  corresponding  to  the  area  of  distribution  of 


224  NK11YOUS  DISEASES 

the  posterior  cerebral  artery.  The  cyst  is  lined  by  a  wall  of 
dense  fibrous  tissue.  The  remainder  of  the  hemisphere  is  in  a 
state  of  sclerotic  atrophy  with  microgyria;  the  convolutions 
are  small  and  hard,  the  sulci  are  wide,  and  the  surface  is  not 
unlike  that  of  the  kernel  of  a  walnut.  Microscopic  examina- 
tion shows  the  almost  complete  disappearance  of  the  nerve 
elements  and  their  replacement  by  dense  neuroglial  tissue. 

The  whole  of  one  hemisphere  ma}'  be  affected,  or  only 
certain  areas ;  the  basal  ganglia  commonly  escape.  The 
blood-vessels  are  of  normal  appearance. 

The  atrophic  area  of  the  brain  may  be  compensated 
partly  by  an  excess  of  the  cerebro-spinal  fluid,  and  partly 
by  aii  increase  of  the  thickness  of  the  calvarium  over  the 
affected  regions. 

Clinically,  the  cases  of  this  condition  are  of  acute  onset. 
They  may  occur  during  intra-uterine  life,  and  result  from 
encephalitis  or  acute  vascular  lesions.  The  picture  presented 
in  the  later  stages  depends  upon  the  situation  and  size  of 
the  lesion,  and  may  present  all  intermediate  forms  between 
infantile  hemiplegia,  diplegia,  and  simple  mental  deficiency. 

Secondary  degenerations  occur  in  the  lower  parts  of  the 
brain  and  spinal  cord  as  a  result  of  the  different  cerebral 
lesions  just  described.  When  the  lesion  affects  mainly  the 
cortical  motor  areas,  the  pyramids  and  the  crossed  and  direct 
pyramidal  tracts  are  either  absent,  ill-developed,  or  degene- 
rated. With  extensive  unilateral  lesions,  involving  both  the 
centrum  ovale  and  the  basal  ganglia,  there  is  a  crossed 
cerebellar  atrophy  and  wasting  of  the  mesial  fillet.  The 
fronto-pontine  and  temporo-pontine  tracts  are  degenerated 
after  lesions  more  or  less  confined  to  the  frontal  and  temporal 
lobes. 

Physical  conformation.  These  patients  are  usually  under- 
grown,  and  present,  in  addition  to  the  deformities  of  the 
limbs  already  described,  other  marked  physical  peculiarities. 
In  the  majority  of  the  diplegic  cases  the  head  is  small, 
though  in  a  few  it  is  larger  than  normal.  In  the  hemiplegic 
cases,  the  skull  over  the  affected  hemisphere  is  smaller  than 
over  the  non-  affected,  leading  to  marked  cranial  asymmetry. 
Other  stigmata  of  degeneration  may  be  seen  in  the  high, 
narrow,  and  deformed  palates  presented  by  some  cases.     The 


DISEASES  OF  THE  BRAIN  225 

body  and  limbs  on  the  paralysed  are  smaller  tban  on  the 
normal  side,  a  change  which  is  due  to  defective  development 
of  all  the  tissues.  The  want  of  growth  is  sometimes  more 
apparent  than  real,  owing  to  contracture  and  deformity,  as 
measurements  fail  to  bring  out  any  definite  shortening  of  the 
bones  of  the  paralysed  limbs. 

Prognosis.  The  prognosis  in  the  cerebral  palsies  of 
infants  and  children  depends  upon  a  number  of  conditions. 
The  diplegic  cases  have,  on  the  whole,  a  shorter  life  than 
the  hemiplegic,  for  it  is  not  common  to  find  such  patients 
living  much  beyond  early  adult  life.  If  the  child  survive  the 
acute  onset  of  the  disease,  even  in  a  partially  paralysed  state, 
life  may  be  prolonged  for  many  years.  The  onset  of  epileptic 
attacks,  however,  introduces  an  element  of  uncertainty  and 
some  danger.  On  the  other  hand,  recovery  from  the  acute 
onset  may  not  occur.  There  are  cases,  however,  in  which 
recovery  is  so  satisfactory  that  it  is  scarcely  possible  to  detect 
any  evidence  of  a  local  lesion.  These  cases,  notwithstanding, 
may  develop  epilepsy  about  the  time  of  puberty. 

Pronounced  athetosis  is  unfavourable  in  so  far  as  it 
interferes  with  a  satisfactory  issue  to  treatment.  Mental 
deterioration,  marked  rigidity,  and  paralysis  are  unfavourable, 
owing  to  the  liability  to  intercurrent  complications. 

Treatment  is  confined  almost  entirely  to  physical  exercises 
with  a  view  to  the  correction  of  motor  disabilities,  rigidity,  and 
contractures,  and  educational  exercises  to  develop  and  improve 
the  mental  condition.  It  is  scarcely  necessary  to  describe 
these  in  detail,  as  they  are  conducted  along  similar  general 
lines  to  those  in  healthy  children.  It  is  preferable  that  these 
children  should  be  instructed  either  in  special  schools  or  under 
private  tuition.  Instruction  in  elocution  is  an  important 
factor  when  speech  defects  are  present. 

Contractures  in  the  feet  are  corrected  by  properly  adjusted 
boots  or  by  tenotomy.  Epileptic  attacks  should  be  controlled 
in  the  manner  described  under  Idiopathic  Epilepsy  (p.  579). 

CEREBELLAR  AFFECTIONS  IN  CHILDREN 

A  condition,  analogous  to  cerebral  diplegia,  may  occur 
from  arrested  development  or  early  death  (agenesia)  of  the 
nerve  elements  of  the  cerebellum.      It  is  usually  associated 

15 


226  NERVOUS  DISEASES 

with  similar  changes  in  the  cerebrum,  but  little  is  known 
about  it. 

Symptoms.  Acute  cerebellar  ataxia  may  come  on  suddenly, 
either  during  the  course  of,  or  shortly  after,  an  acute  febrile 
disorder.  There  are  other  cases,  also,  in  which  similar 
symptoms  arise  without  any  antecedent  disease. 

Although  there  are  no  certain  pathological  data  that  these 
cases  are  due  to  inflammatory  or  vascular  lesions,  such  as 
have  been  already  described  in  the  cerebrum,  yet  presumptive 
evidence  exists  to  show  that  they  may  be  due  to  : — 

(a)  Encephalitis  affecting  the  cerebellum  and  its  mesen- 
cephalic connexions. 

(b)  Acute  vascular  lesions  (hemorrhage  or  thrombosis) 
arising  either  during  or  after  an  acute  specific  disease. 

These  affections  are  characterised  clinically  by  inco-ordi- 
nation  of  the  movements  of  the  trunk,  head,  neck,  limbs, 
and  eyes,  and  of  articulation.  In  some  cases  there  is 
evidence  that  the  disease  simultaneously  affects  other  areas — 
such  as  the  midbrain  and  cerebrum.  In  one  case  personally 
observed  (following  upon  measles),  nystagmus,  slight  arti- 
culatory  inco-ordination,  ataxia  of  the  upper  limbs — but 
especially  of  the  trunk  and  lower  limbs — were  present.  The 
motor,  sensory,  and  reflex  functions  showed  no  evidence  of 
affection  of  any  part  of  the  brain  other  than  the  cerebellum. 
The  mental  condition  was  unaffected. 

The  prognosis  in  these  cases  is  good,  a  slow  and  gradual 
improvement  taking  place.  The  ataxia  of  the  trunk  and 
lower  limbs,  when  standing  or  walking,  is  the  last  symptom 
to  disappear.  The  more  definitely  limited  the  symptoms 
are  to  the  cerebellum,  the  more  hopeful  is  the  outlook 
for  recovery. 

Chapter  V 
INTRACRANIAL  TUMOURS 

The  advances  which  have  been  made  in  the  localisation  of 
intracranial  tumours,  and  in  the  knowledge  of  their  nature  and 
mode  of  growth,  have  placed  their  treatment  within  the  range 
of  practical  surgery.  Many  of  these  growths,  either  from  their 
position,  their  character,  or  their  size,  cannot  be  extirpated ; 


DISEASES  OF  THE  BRAIN  227 

but  even  in  such  cases  the  employment  of  surgical  interference 
as  a  palliative  rather  than  a  radical  measure  may  be  of  the 
greatest  benefit  in  relieving  the  general  symptoms,  preventing 
blindness,  and  in  prolonging  life. 

Feequency  and  Chaeactee  op  Tumoues 

The  relative  frequency  of  the  various  forms  of  tumour 
growth  affecting  the  brain  differs  materially  according  as 
statistics  are  drawn  from  children  or  adults.  This  difference 
is  so  marked  that  to  give  a  collective  table  is  entirely 
misleading. 

In  children,  tuberculous  tumours  are  as  common  as  all 
other  forms  of  new  growth  taken  together.  Gliomata  are 
next  most  frequent ;  then  the  sarcomata,  and  then  miscel- 
laneous forms  of  tumour.  In  adults,  gliomata  form  48  per 
cent.,  sarcomata  31  per  cent.,  tubercle  6  per  cent.,  endothelio- 
mata  5*5  per  cent.,  carcinomata  5  per  cent.,  gummata  3  per 
cent.,  and  tumours  of  the  choroid  plexus  1-5  per  cent.  These 
percentages  are  drawn  from  the  annexed  table,  which  is 
based  upon  the  post-mortem  records  of  the  Queen  Square 
Hospital.  These  figures  are  mainly  from  adult  cases,  but 
include  a  small  percentage  of  children. 


Single. 

Multiple. 

Total. 

Glioma 

95 

1 

96 

Sarcoma  . 

52 

4 

56 

Endothelioma 

11 

0 

11 

Tubercle  . 

8 

4 

12 

Fibro-sarcoma  . 

6 

0 

6 

Gumma    . 

4 

2 

6 

Carcinoma 

2 

8 

10 

Tumours  of  the 

choroid  plexn 

s         3 

0 

3 

Totals  181  19  200 

New  growths  within  the  brain  may  be  primary  or  secondary 
to  tumour  growth  elsewhere. 

Gliomata.  The  gliomata  are  the  most  common,  and  may 
occur  at  any  age.  They  are  rarely  multiple.  They  may  be 
situated  in  any  part  of  the  brain.    They  are  of  an  infiltrating 

15  * 


228  NERVOUS  DISEASES 

character.  Several  varieties  are  found:  (a)  a  small-celled, 
more  or  less  circumscribed  growth ;  (b)  a  large-celled, 
vascular,  rapidly  growing  and  infiltrating  tumour;  (c)  forms 
of  an  intermediate  type. 

Those  of  rapid  growth  tend  towards  cystic  degeneration, 
especially  when  situated  in  the  cerebellum,  and  are  not 
infrequently  the  seat  of  hemorrhage.  They  do  not  implicate 
the  meninges,  or  bones  of  the  skull.  They  may  be  seen 
spreading  along  small  blood-vessels,  and  at  the  margin  of  the 
tumour  small  islands  of  new  growth  are  observed  surrounded 
by  normal  tissue.  Where  c}^stic  degeneration  has  taken  place, 
neuroglial  cells  may  be  seen  arranged  in  several  layers 
radiating  outwards  from  the  cavity.  In  the  pons,  where 
glioma  occurs  in  the  form  of  '  hypertrophy  of  the  pons,' 
a  wide  infiltration  of  glial  cells  is  seen,  without  much 
tendency  to  cystic  degeneration. 

Sarcomata  arise  in  connexion  with  the  meninges,  periosteum, 
blood-vessels,  and  bones.  They  are  found  at  all  ages.  They 
involve  the  brain  tissue  indirectly,  and  destroy  the  cerebral 
tissue  by  compression.  They  also  radiate  and  extend  along 
the  cerebral  blood-vessels.  They  are  more  often  multiple  than 
the  gliomata.  When  growing  from  the  meninges  or  periosteum 
they  are  fibro- sarcomatous  in  character,  are  usually  of  a  dense, 
firm  consistence,  and  often  attain  to  large  size. 

Eound- celled  sarcomata  are  seen  in  connexion  with  the 
soft  membranes  and  blood-vessels,  and  tend  towards  greater 
malignancy  than  the  fibrous  variety.  Melanotic  sarcoma, 
always  a  secondary  tumour,  is  the  most  malignant  type,  and 
may  be  scattered  throughout  the  brain,  or  widely  spread  over 
the  meninges.  Sarcomata,  growing  from  the  base  of  the  skull, 
may  attain  a  large  size  and  be  very  widely  diffused. 

Tuberculous  tumours  may  be  solitary  or  multiple.  They 
may  be  situated  deeply  or  on  the  surface.  They  are  found 
especially  in  children.  The  tumours  are  firm  and  non- 
vascular, tending  towards  caseation  and  sometimes  purulent 
disintegration.  The  tubercle  bacillus  can  be  demonstrated  in 
most  instances.  They  may  remain  quiescent  for  long  periods, 
or  give  rise  suddenly  to  tuberculous  meningitis. 

Endutheliomata  spring  from  the  dura  mater  and  extend  on 
the  one  hand  into  the  skull  and  on  the  other  hand  into  the 
brain.     They  do  not  infiltrate  the  brain,  but  push  it  before 


DISEASES  OF  THE  BRAIN  229 

them  and  compress  the  nerve  fibres.     They  may  occur  at  any 
age,  are  always  single,  and  may  attain  to  a  large  size. 

Gummata  are  sometimes  single,  but  more  usually  multiple, 
or  associated  with  gummatous  meningitis.  They  invariably 
grow  from  the  membranes.  Commonly  situated  on  the  surface 
of  the  brain,  they  may  be  found,  however,  in  the  central  parts 
growing  from  one  of  the  deep  folds  of  pia  mater.  They  tend 
either  to  break  down,  or  to  form  firm  fibrous  granulomatous 
masses.  They  do  not  suppurate.  The  surrounding  brain  is 
destroyed  partly  by  pressure,  and  partly  by  vascular  occlusion 
and  inflammatory  exudation. 

Psammomata  are  found  in  connexion  with  the  meninges 
and  the  pineal  gland.     They  do  not  attain  to  a  large  size. 

Adenomata  may  arise  in  connexion  with  the  hypophysis 
cerebri  (pituitary  body) . 

Tumours  of  the  choroid  plexus  are  carcinomatous  with  a 
papillomatous  structure.  They  arise  from  the  choroid  plexus 
and  are  situated  inside  the  ventricles,  but  may  infiltrate  the 
surrounding  tissue,  or  extend  through  the  ventricular  openings 
and  appear  upon  the  outer  surface  in  the  lateral  recesses. 
Occasionally  they  give  rise  to  secondary  deposits  in  the  central 
canal  of  the  spinal  cord,  on  the  surface  of  the  cord,  or  on  the 
posterior  nerve  roots. 

Carcinomata.  The  carcinomata  are  soft,  often  vascular 
and  irregular  in  outline  and  distribution.  They  are  found 
growing  from  the  membranes,  or  deeply  in  the  brain 
substance.  They  are  usually  multiple,  and  are  more  or  less 
circumscribed.  Of  our  ten  cases  the  tumours  were  secondary 
to  primary  growths  in  the  breast  (three  cases),  rectum  (four 
cases),  supra-renal  capsules  (two  cases),  and  lung  (one  case). 

Parasitic  cysts  are  of  two  varieties — the  cysticercus  and 
the  echinococcus.  They  are  rare  in  this  country.  They 
occur  more  commonly  in  connexion  with  the  ventricles  of  the 
brain. 

Situation  op  Tumoues 

Cerebrum.  Cerebral  tumours  are  more  common  in  adults 
than  cerebellar  or  pontine  tumours.  In  children,  on  the  other 
hand,  pontine  and  cerebellar  tumours  are  more  frequent,  the 
cerebrum  being  rarely  affected  except  by  tuberculous  tumours. 

The  frontal  area  is  rarely  the  seat  of   tumour  in  young 


230  NERVOUS  DISEASES 

children.  Glioma  is  the  most  frequent  form  in  this  locality, 
and  usually  develops  between  the  ages  of  thirteen  and  thirty- 
five.    Fibrosarcoma  develops  in  adult  and  later  life. 

Glioma  is  the  most  common  deep-seated  tumour  in  the 
other  regions  of  the  cerebrum.  The  sarcomata  are  next  in 
frequency  and  arise  later  in  life,  and  involve  the  cortex 
more  often  than  the  deeper  tissues.  The  gummata  and  the 
carcinomata  are  usually  cortical,  often  multiple,  and  arise 
in  adult  and  later  life  respectively. 

Endothelioma  occurs  in  early  and  mid-adult  life,  grows 
from  the  membranes,  and  does  not  infiltrate  the  brain  tissue. 

Cerebellum.  This  region  is  commonly  affected  in  children  by 
tubercle,  glioma,  and  sarcoma.  In  adults,  gumma,  sarcoma, 
and  carcinoma  are  the  most  common  varieties  of  new  growth. 

Pons  Varolii.  The  glioma  is  the  most  common  form  of 
tumour.  It  is  found  in  children  and  young  adults.  Tubercle 
is  also  common  in  children. 

Fourth  ventricle.  Gliomata,  sarcomata,  papillomata,  and 
parasitic  cysts  may  be  found.  The  papillomata  grow 
from  the  choroid  plexus.  These  tumours  may  occur  at 
any  age. 

Extra- cerebellar  tumours  do  not  occur  in  young  children, 
but  from  the  age  of  fifteen  years  onwards.  They  are  usually 
fibro-sarcomata  or  neuro-fibromata. 

The  Table  on  page  231  shows  the  relative  frequency 
of  the  different  kinds  of  tumour  in  the  several  regions  of 
the  brain. 

Anatomical  and  Physiological  Factoks 

In  order  to  obtain  a  fuller  comprehension  of  the  general 
effects  of  intracranial  tumours,  reference  will  be  made  to  some 
anatomical  and  physiological  factors,  which  play  an  important 
role  in  their  production. 

The  brain  is  enclosed  in  the  cranial  box,  from  which  there 
is  only  one  large  outlet — the  foramen  magnum,  through 
which  the  brain  is  in  continuation  with  the  spinal  cord. 

The  brain  is  a  viscous  mass  completely  filling  the  cranial 
cavity.  It  is  surrounded  by  three  membranes :  (1)  The  pia 
mater.  This  is  in  close  connexion  with  the  surface  of  the  brain, 
dips   into  the  sulci,  and  sends  processes  into   the    cerebral 


DISEASES  OF  THE  BRAIN 


231 


Kelative  Frequency  of  the  Different  Kinds  of  Tumour  in  the  Several 
Kegions  of  the  Brain. 


■i 

6 
3 

o 
in 

Tubercle. 

i 

o 

a 

a 
n 

H 
O 
0 
y. 

H 

Frontal  area    . 

14 

5 

— 

3 

1 

1 

24 

Kolandic     „     . 

2 

8 

— 

1 

1 

1 

13 

Parietal       ,,     . 

6 

— 

— 

1 

1 

— 

8 

Occipital      .     . 

2 

2 

— 

— 

— 

— 

4 

Temp,  sphen.  . 

9 

9 

— 

— 

— 

— 

18 

Corp.  callos 

2 

1 

— 

— 

— 

— 

3 

Centr.  ovale     . 

8 

1 

— 

— 

— 

— 

9 

Basal  ganglia  . 

5 

2 

1 

— 

— 

— 

8 

Mesencephalon 

8 

4 

2 

— 

— 

— 

14 

Pons  Varolii    . 

17 

— 

— 

— 

— 

— 

17 

Cerebellum  .     . 

17 

8 

5 

— 

1 

— 

31 

Extracerebellar 

1 

5 

— 

1 

— 

4 

11 

Latl.  ventricle 

2 

— 

— 

— 

— 

— 

2 

Fourth  ventricle 

1 

2 

— 

— 

— 

— 

3 

Insula          .     . 

1 

— 

— 

— 

— 

— 

1 

Falx  .... 

— 

— 

— 

5 

— 

— 

5 

Pituitary  body 

— 

2 

— 

— 

— 

- 

2 

Tentorium  .     . 

— 

1 

— 

— 

— 

— 

1 

Skull     .      .     . 

— 

2 

— 

— 

— 

— 

2 

Total  .     . 

95 

52 

8 

11 

4 

6 

176 

Tumours  of  the  choroid  plexus  (third  and  fourth  ventricles)  and  the  carcinomata 
are  omitted. 


232  NERVOUS  DISEASES 

substance.  (2)  The  arachnoid  mater.  This  is  somewhat 
thicker,  is  loosety  connected  to  the  pia,  and  bridges  over  the 
sulci  between  the  convolutions.  (3)  The  dwra  mater:  a  dense, 
strong,  sensitive  membrane  in  close  relation  with  the  skull. 
^Yithin  the  dura  are  situated  the  cerebral  venous  sinuses  into 
which  the  blood  from  the  cerebral  veins  passes  on  its  way  to 
the  emissary  veins,  which  convey  it  from  the  cranial  cavity. 

The  dura  mater  sends  processes  into  the  cranial  cavity, 
dividing  it  into  three  more  or  less  distinct  chambers.  In  the 
first  place,  the  tentorium  cerebelli  cuts  off  the  posterior  fossa 
from  the  anterior  and  middle  fossa\  This  is  a  particularly 
strong  and  resistant  membrane,  with  only  one  opening  in  it, 
which  is  completely  filled  by  the  crura  cerebri  connecting  the 
cerebrum  with  the  pons.  In  the  posterior  chamber,  beneath 
the  tentorium,  lie  the  pons,  cerebellum,  and  bulb.  Secondly, 
the  cerebral  chamber,  which  is  above  the  tentorium,  is  divided 
into  two  halves  by  the  falx  cerebri,  a  process  of  dura  mater 
which  lies  in  the  medial  sagittal  plane,  and  separates  the  two 
cerebral  hemispheres  in  front  and  above  the  corpus  callosum. 
This  subdivision  of  the  cerebral  chamber  is  therefore  much 
less  complete,  owing  to  the  large  size  of  the  corpus  callosum  and 
the  relative  laxity  of  the  partition  between  the  two  hemispheres. 

Under  the  dura  and  arachnoid  membranes  are  the  sub- 
dural and  subarachnoid  spaces,  which  in  normal  conditions 
are  merely  potential  cavities,  but  contain  a  small  amount  of 
cerebro-spinal  fluid.  These  spaces  communicate  with  the 
subarachnoid  spinal  space  and  with  the  intraventricular 
system.  The  dura  and  arachnoid  membranes  are  also 
reflected  along  the  optic  nerves  to  form  the  optic  sheaths  and 
perineurium,  so  that  the  subdural  and  subarachnoid  spaces 
are  continued  along  the  optic  nerves  outside  the  cranial  cavity. 

Inside  the  brain  is  a  ventricular  system  consisting  of  the 
two  lateral  ventricles — one  in  each  cerebral  hemisphere — 
communicating  by  the  foramina  of  Monro  with  the  third 
ventricle,  which  is  mesial  and  basal  in  position.  From  the 
hinder  end  of  the  third  ventricle  the  aqueduct  of  Sylvius 
passes  downwards  into  the  fourth  ventricle,  which  lies  in  the 
posterior  chamber  between  the  cerebellum,  the  pons,  and  the 
upper  portion  of  the  medulla  oblongata. 

The  fourth  ventricle  communicates  with  the  subarachnoid 
space  by  the  foramina  of  Majendie  and  by  openings  in  the 


DISEASES  OF  THE  BRAIN  233 

lateral  recesses  of  the  ventricle  between  the  medulla  and  the 
cerebellum. 

The  subdural  and  subarachnoid  spaces  of  the  brain  and 
cord  are  directly  continuous  through  the  foramen  magnum. 

The  Cerebral  Circulation 

The  arterial  supply  of  the  brain  is  provided  by  two  sets  of 
vessels,  the  internal  carotid  and  vertebral  arteries,  which  anas- 
tomose freely  (p.  166).  There  are  no  valves  in  the  cerebral 
veins,  and  there  is  no  lymphatic  system  apart  from  the  vascular 
system.  This  is  provided  with  large  perivascular  spaces,  and, 
probably  also  with  small  intra- adventitial  spaces,  which 
subserve  the  functions  of  a  lymphatic  system.  In  normal 
conditions  the  arterial  pulsation  is  transmitted  to  the  cerebral 
sinuses  (Hill). 

The  brain  expands  within  the  cranial  cavity.  The  expan- 
sion is  of  two  kinds  :  (1)  circulatory  with  arterial  pulsation, 
and  (2)  respiratory.  The  experiments  of  Leonard  Hill1  show 
that  the  brain  stands  in  such  close  relationship  to  the  general 
venous  pressure,  that  its  greatest  expansion  is  during  expira- 
tion, owing  to  the  rise  of  pressure  which  takes  place  in  the 
right  side  of  the  heart.  This  dams  back  the  blood  into  the 
cerebral  sinuses  and  forces  the  cerebro-spinal  fluid  from  the 
cranium  into  the  less  rigid  vertebral  canal.  During  inspira- 
tion, on  the  other  hand,  the  blood  flows  from  the  cerebral  veins 
and  the  cerebro-spinal  fluid  returns  into  the  skull. 

There  is  a  definite  relationship  between  the  intracranial 
venous  pressure  and  that  of  the  cerebro-spinal  fluid.  The 
former  cannot  remain  higher  than  the  latter,  as  transudation 
takes  place  from  the  veins  into  the  cerebro-spinal  fluid  until 
equilibrium  is  restored.  Thus  a  rise  in  intracranial  venous 
pressure  implies  a  rise  in  the  cerebro-spinal  fluid  pressure. 
The  withdrawal  of  cerebro-spinal  fluid  therefore  can  only  be 
of  temporary  benefit  in  the  relief  of  increased  intracranial 
tension.  The  one  method  of  relieving  intracranial  pressure 
is  to  reduce  the  venous  pressure  within  the  skull  either  by 
venesection,  purgation,  or  by  removing  a  sufficiently  large 
portion  of  the  calvarium. 

In  tumour  of  the  brain  the  increased  intracranial  tension 
is  due  to  an  increase  of  the  contents  of  the  skull  which  causes 

1  Leonard  Hill  The  Cerebral  Circulation,  1S96 


L'.-U 


NERVOUS  DISEASES 


an  obstruction  to  the  cerebral  circulation,  thus  raising  the 
venous  pressure  and,  pari  passu,  that  of  the  cerebro-spinal 
fluid.     (See  under  Cerebral  Hemorrhage,  p.  196.) 

"When  the  general  intracranial  pressure  is  raised,  the  brain 
is  pressed  against  the  dura  mater  and  the  cerebro-spinal  fluid  is 


Pig.  72. — Represents  a  tumour  of  the  left  frontal  lobe  growing 
across  the  mesial  plane,  and  secondarily  affecting,  by  pressure, 
the  right  hemisphere. 


forced  from  the  cranial  subdural  and  subarachnoid  spaces  into 
the  corresponding  spinal  spaces  and  the  optic  nerve  sheaths. 

The  determining  factors  in  the  production  of  the  general 
effects  of  intracranial  tumour  therefore  are  :  (1)  the  situation 
and  size  of  the  tumour ;  (2)  the  nature  and  rate  of  growth 
of  the  tumour ;  (3)  the  local  effect  of  the  tumour  upon  the 
brain  substance.  The  focal  effects  depending  upon  the 
local  lesion  will  be  discussed  later  under  Focal  Diagnosis. 


DISEASES  OF  THE  BRAlN 


235 


Effects  of  Intracranial  Tumours  upon  the  Cranial  Contents 

1.  Tumours  situated  in  the  anterior  or  cerebral  chambers. 
A  tumour  in  one  or  other  of  the  anterior  cerebral  chambers 
gives  rise  to  the  following  sequence  of  events. 


Fig.  73.  — Shows  "the  ventral  aspect   of  the   brain  with  a  tumour  Jinvolvins 
the  frontal  lobes. 


In  the  first  place,  there  is  an  increase  in  the  bulk  of  the 
contents  of  the  chamber,  with  the  result  that  a  rise  of 
pressure  occurs  in  that  chamber.  Secondly,  this  is  followed 
by  a  rise  of  pressure  in  the  opposite  anterior  chamber 
and  a  consequent   increase   of  the  supra-tentorial  tension ; 


L>3(i 


NERVOUS  DISEASES 


and  thirdly,  by  a  rise  of  the  general  intracranial  pressure 
both  above  and  below  the  tentorium. 

These  general  results  are  dependent  upon  an  actual 
increase  in  the  total  solid  bulk  of  the  cranial  contents ;  and  if 
the  brain  be  a  constant  quantity,  the  larger  the  tumour  the 
greater  the  pressure.  But  if  the  tumour  grows  at  the  expense 
of  the  brain  substance  and  destroys  it,  then  the  pressure  is 


Fig.  74. —  .Shows   the    formation    of   a   'pressure    cone,'   photographed  from 

the  side. 


lessened  proportionately  to  the  amount  of  brain  substance 
which  is  destroyed. 

The  constant  effect  is  to  displace  the  brain  from  the  seat 
of  tumour  in  the  direction  of  least  resistance ;  and  this  effect 
is  first  felt  in  the  chamber  in  which  the  growth  is  situated. 
The  brain  may  be  displaced  upwards,  downwards,  forwards, 
backwards,  or  across  the  middle  line.  The  skull  being 
practically  unyielding,  the  nerves  which  run  over  the  base 
of  the  brain  may  suffer  from  direct  compression  ;  on  the  other 
hand,  the  points  of  least  resistance  are  the  ventricles  and  the 
opposite  hemisphere,  and  therefore  the  affected  hemisphere 
tends  to  be  displaced  across  the  middle  line.  Thus  a  rise  of 
pressure  in  both  anterior  chambers  takes  place,  and  eventually 


DISEASES  OF  THE  BRAIN 


2.'57 


becomes  so  great  that  the  tentorium  cerehelli  is  displaced 
downwards  and  a  '  pressure  cone '  is  formed  by  the  blocking 
of  the  foramen  magnum  by  the  bulb.     (Figs.  74  and  75.) 

These  stages,  in  the  development  of  the  increased  intra- 
cranial pressure,  may  be  recognised  clinically,  in  the  first  stage, 
by  the  earlier  occurrence  of  optic  neuritis  on  the  same  side  as 
the  lesion  in  cerebral  as  contrasted  with  cerebellar  growths ; 


Fig.  75. 


-Shows  a  'pressure  cone'  affecting  the  medulla  oblongata,  viewed 
from  behind. 


and  by  the  signs  of  interference  with  the  functions  of  the 
same  cerebral  hemisphere — such  as  changes  in  the  reflexes 
and  slight  hemiparesis  on  the  opposite  side  of  the  body — apart 
from  the  immediate  local  effects  of  the  growth.  The  second 
stage,  when  the  increased  pressure  affects  both  cerebral  hemi- 
spheres, is  demonstrated  by  the  onset  of  mental  changes  and 
by  evidence  of  impairment  of  function  of  the  opposite  hemi- 
sphere, so  that  changes  in  the  reflexes  are  found  on  both  sides. 
This  accounts  for  the  earlier  onset  of  the  mental  symptoms  in 
cerebral  than  in  cerebellar  tumours.  The  third  stage  is 
characterised  by  the  failure  of  the  respiratory  centre  from 
anaemia  of  the  bulb  owing  to  the  formation  of  a  '  pressure 
cone.' 


238  NERVOUS  DISEASES 

2.  Tumours  sit  netted  in  the  posterior  or  cerebellar  chamber 
— subtentorial  tumour*. 

The  posterior  chamber  is  smaller  than  the  anterior,  its 
iloor  is  formed  by  the  posterior  fossa  of  the  skull,  its  sides 
and  roof  by  the  occipital  bones  and  the  firm  resisting 
tentorium  cerebelli.  The  place  of  least  resistance  is  the 
foramen  magnum. 

Tumours  in  the  posterior  chamber  may  be  situated  :  (1) 
outside  the  cerebellum,  pons,  or  medulla ;  and  (2)  inside  the 
cerebellum,  pons,  medulla,  or  fourth  ventricle. 

Of  tumours  situated  outside  the  cerebellum,  the  most 
common  are  those  in  connexion  with  the  auditory  nerves.  As 
a  rule  they  are  of  slow  growth  and  firm  consistence,  and  tend 
to  grow  at  the  expense  of  the  nervous  tissue  of  the  pons  and 
cerebellum,  which  become  indented  and  atrophied  by  the 
constant  pressure  upon  them.  It  is  therefore  not  until 
the  growth  has  attained  a  sufficient  size  to  force  the  bulb 
down  into  the  foramen  magnum  that  any  great  increase  in 
intracranial  tension  takes  place.  When  this  occurs  the  com- 
munication between  the  cranial  and  vertebral  cerebro-spinal 
spaces  is  cut  off.  In  consequence,  a  rise  occurs  in  the 
intraventricular  and  intracranial  tension  with  resultant  optic 
neuritis,  provided  the  patient  survives  the  effects  of  the 
bulbar  anaemia.  Clinically,  we  find  in  these  cases  early, 
well-defined,  and  long-continued  local  symptoms,  with  a  late 
onset  and  rapid  development  of  the  general  symptoms  of 
intracranial  tumour  and  often  sudden  death. 

In  tumours  growing  within  the  cerebellum  displacement 
is  towards  the  points  of  least  resistance — the  fourth  ventricle 
and  the  foramen  magnum.  In  some  cases  the  ventricular 
system  is  obstructed  with  a  consequent  rise  in  the  intraven- 
tricular tension.  In  other  cases  there  is  only  partial  blocking, 
with  pressure  exerted  downwards  upon  the  bulb,  and  inter- 
ference with  the  communication  between  the  cranial  and 
vertebral  subdural  and  subarachnoid  spaces.  Increasing  rise 
of  pressure  leads  to  anaemia  of  the  vital  medullary  centres. 

Tumours  growing  into  or  in  the  fourth  ventricle  may 
extend  and  appear  externally  through  the  lateral  recess 
between  the  medulla  and  cerebellum,  and  may  eventually, 
along  with  the  medulla,  form  part  of  the  '  pressure  cone.' 

In  these  cases  the  ventricular  system  becomes  distended, 


DISEASES  OF  THE  BHAIN  239 

the  subarachnoid  and  subdural  spaces  obliterated,  and  optic 
neuritis  develops  early  and  simultaneously  in  both  eyes. 
Mental  symptoms  are  not  common  in  cases  of  cerebellar 
tumour :  as  a  rise  in  intracranial  tension,  sufficient  to  cause 
compression  and  interference  with  cerebral  function,  is  not 
compatible  with  life. 


Fig.  76. — -Shows  a  glioma  of  the  right  cerebellar  lobe  which  has  undergone 
cystic  degeneration.  It  is  displacing  the  middle  lobe  and  pressing  into 
the  fourth  ventricle. 


Stupor  and  coma,  therefore,  are  associated  with  respiratory 
failure  as  terminal  symptoms. 

The  tendency  for  tumours  in  the  posterior  chamber  to 
give  rise  to  internal  hydrocephalus  is  in  strong  contrast  to 
what  is  seen  in  tumours  of  the  anterior  chambers.  Cystic 
gliomas  are  of  much  more  frequent  occurrence  in  the  cere- 
bellum than  in  the  cerebrum,  and  this  may  be  partly  explained 
by  the  smallness  of  the  posterior  chamber  and  the  consequent 
earlier  cutting  off  of  the  blood  supply  to  the  growth,  with 
resulting  necrosis  and  cystic  formation.  The  effect  of  such 
cystic  formation  is  to  reduce  the  intracerebellar  tension  and 


240  NERVOUS  DISEASES 

alleviate  the  pressure  symptoms  with  re-establishment  of  the 
ventricular  and  subarachnoid  communication.     (Fig.  7G.) 

It  is  obvious  that  lumbar  puncture  as  a  therapeutic 
measure  in  such  cases  cannot  do  good,  and  may  possibly  even 
do  harm  by  lessening  the  spinal  resistance  in  the  foramen 
magnum  and  permitting  the  further  downward  displacement 
of  the  bulb. 

Tumours  of  the  pons,  if  of  an  infiltrating  or  gliomatous 
nature,  are  slowly  progressive,  and  only  give  rise  to  increased 
intracranial  tension  by  obstructing  the  intraventricular  system. 
In  these  cases  there  is  a  gradual  rise  in  the  intraventricular 
tension :  the  ventricle  most  affected  being  the  third,  which  is 
the  weakest  of  the  supra-tentorial  series.  Its  distension, 
therefore,  may  result  in  direct  pressure  upon  the  optic  tracts 
and  chiasma.  This  may  account  in  part  for  the  absence  of 
optic  neuritis  in  such  cases,  and  the  not  infrequent  occurrence 
of  primary  optic  atrophy. 

Tumours  of  whatever  nature  may  have  a  iatal  effect  from 
their  situation ;  but  such  cases  are  rare,  the  true  cause  of 
death  being  ancemia  of  the  cardiac  and  respiratory  centres. 

The  above  facts  explain  in  large  part  the  origin  of  the 
so-called  false  localising  signs,  which  are  due  to  indirect 
pressure  effects. 

General  Symptomatology 

Headache.  This  is  a  more  or  less  constant  feature,  varying 
in  character  from  a  dull  aching  to  a  sharp,  radiating  pain. 
It  may  come  and  go  quite  suddenly.  Change  of  posture  or 
movement  of  the  head  may  bring  it  on.  Frequently  it  is 
associated  with  a  feeling  of  nausea  or  with  attacks  of  vomiting. 
When  the  pain  is  localised  to  one  particular  spot,  which  is 
tender  on  pressure,  it  is  evidence  in  favour  of  the  growth 
being  situated  on  the  surface  of  the  brain,  or  growing  from 
the  dura  mater.  The  chief  regions  where  general  head- 
ache is  complained  of  are  behind  the  eyes,  in  both  temples, 
and  over  the  vertex.  Occipital  pain  is  suggestive  of  a  growth 
in  the  posterior  fossa,  and  unilateral  frontal  pain  of  one  in  the 
frontal  region. 

Vomiting-.  This  may  or  may  not  develop  till  late  in  the 
history  of  the  case.     In  its  most  characteristic  form,  it  is 


DISEASES  OF  THE  BRAIN  241 

associated  with  headache,  and  is  independent  of  the  taking  of 
food.  There  is  no  pain  or  retching,  and  the  patient  has  no 
nausea  afterwards.  Vomiting  is  less  frequent  than  headache, 
but  the  majority  of  cases  suffer  from  attacks  of  vomiting,  often 
brought  on  by  sudden  change  of  posture  or  sudden  increase 
in  the  intracranial  pressure. 

Optic  neuritis.  The  time  at  which  optic  neuritis  may 
develop  is  variable,  as  already  explained  (p.  237).  As  a 
general  rule  optic  neuritis  occurs  early,  and  is  very  intense 
in  cases  of  intracerebellar  and  intraventricular  growths.  In 
cerebral  tumours  its  onset  is  more  gradual,  and  in  extra- 
cerebellar  and  intrapontine  tumours  the  patient  may  die 
before  any  swelling  of  the  disc  has  become  visible.  There  is 
little  relation  between  the  situation  of  the  tumour  and  the 
side  on  which  the  optic  neuritis  commences  or  is  more  intense. 
In  cerebral  tumours,  especially  of  the  frontal  area,  it  more 
frequently  commences  first  in  the  eye  on  the  same  side 
as  the  lesion,  from  which  circumstance  it  may  be  of  some 
localising  value. 

A  high  degree  of  swelling  is  quite  compatible  with  good 
vision,  and  in  many  cases  it  is  the  ophthalmoscopic  examina- 
tion which  first  reveals  its  presence.  The  subjective  sensations, 
of  which  complaint  may  be  made,  are  mistiness  before  the 
eyes — often  intermittent — and  later  on  transient  attacks  of 
blindness.  Permanent  loss  of  vision  from  optic  neuritis 
only  commences  when  secondary  atrophic  changes  take  place. 
As  the  swelling  subsides,  the  resulting  fibrous  tissues  contract 
and  give  rise  to  degeneration  of  the  nerve  fibres.  For  this 
reason  the  longer  the  neuritis  lasts,  the  more  hopeless  does 
the  chance  of  the  preservation  of  vision  become. 

Convulsive  attacks.  In  addition  to  the  general  symp- 
toms, already  described,  certain  others  are  of  common 
occurrence,  and  may  be  either  of  general  or  focal  importance. 
Not  infrequently,  patients  suffering  from  intracranial  tumour 
are  subject  to  various  forms  of  seizures.  These  may  be 
'  faints,'  momentary  losses  of  consciousness,  local  convulsive 
movements,  or  generalised  convulsive  attacks.  Such  seizures 
may  simulate  idiopathic  epilepsy ;  and  in  no  case  should 
epilepsy  be  diagnosed  until  the  possibility  of  cerebral 
tumour  has  been  excluded.  In  a  general  convulsive  seizure 
the  patient  falls  down  unconscious,  becomes  rigid  with  tonic 

16 


'2  [-2  NERVOUS  DISEASES 

spasm,  which  passes  into  a  fine  and  rapid  clonic  stage,  and 
terminates  by  clonic  jerkings,  which  become  more  violent  and 
less  frequent  as  the  lit  passes  away.  In  such  attacks  the  face 
may  first  be  pale  and  then  livid.  During  the  clonic  stage  the 
tongue  may  be  bitten.  It  is  well  to  state  here  that  not  only 
may  general  convulsions  occur  as  a  symptom  of  cerebral 
tumour,  but  also  minor  or  '  petit  mal '  attacks. 

A  focal  or  Jacksonian  seizure  is  characterised  by  clonic 
movements  commencing  in  a  definite  region  of  the  body,  and 
either  confined  to  that  region  or  spreading  from  it  in  a 
systematic  order  — 'march  of  the  spasm.'  Consciousness  is 
not  lost  until  the  attack  becomes  generalised.  Such  focal 
attacks  may  have  a  sensory  or  a  motor  commencement, 
dependent  upon  an  irritative  '  discharge '  from  corresponding 
cortical  centres. 

The  importance  of  focal  seizures  will  be  pointed  out  in 
the  account  of  the  localising  symptoms.  They  have  their 
greatest  diagnostic  significance  when  occurring  early  in  the 
history  of  the  case,  but  when  occurring  late  may  be  mis- 
leading and  of  false  localising  value. 

The  mode  of  onset  of  the  focal  seizures  is  of  the  utmost 
importance,  and,  failing  this,  the  observation  of  the  post- 
convulsive or  exhaustion  paralysis. 

Vertigo.  Giddiness  is  not  an  uncommon  symptom,  and 
often  accompanies  attacks  of  headache  and  vomiting.  Its 
significance  as  a  localising  symptom  will  be  considered 
later. 

The  mental  state.  Psychical  symptoms  are  present  in 
most  cases  of  supra-tentorial  tumour.  The  general  mental 
symptoms,  arising  probably  from  the  increase  of  intracranial 
tension  which  occurs  in  the  later  stages  of  the  disease,  are 
dullness,  apathy,  and  irritability  of  temper,  which  may  be 
followed  by  or  develop  into  lethargy,  stupor,  and  eventually 
coma.  On  the  other  hand,  it  is  obvious  that  tumours  situated 
in  the  higher  psychical  centres,  or  association  areas,  may  be 
productive  of  psychical  symptoms  as  early  signs  of  localising 
value,  but  which  are  eventually  obscured  by  those  characteristic 
of  the  terminal  stages  of  all  intracranial  new  growths.  The 
absence  of  early  mental  changes  in  sub-tentorial  tumours  is 
in  striking  contrast  to  their  early  development  and  persistence 
in  cases  of  supra-tentorial  growths. 


DISEASES  OF  THE  BRAIN  243 

TUMOURS  OF  THE  FRONTAL  REGION 

This  region  is  defined  as  that  portion  of  the  brain  lying 
anterior  to  the  ascending  frontal  convolution.     (Fig.  1.) 

The  general  symptoms  of  intracranial  tumour  are  present. 
Headache  is  usually  frontal,  but  not  infrequently  occipital  in 
position.  When  the  tumour  is  near  the  surface  of  the  brain, 
localised  tenderness  on  cranial  pressure  may  be  present. 
Optic  neuritis  may  be  late  in  appearing.  As  a  rule  it 
commences  first,  and  has  its  greatest  intensity  in  the  eye 
on  the  side  of  the  tumour. 

The  mental  symptoms  are  of  special  importance.  They 
are  often  slight  in  degree,  but  are  of  early  onset  and  precede 
the  general  and  the  localising  symptoms.  The  changes  noted 
are  inattention,  inability  to  keep  the  mind  fixed  on  any 
subject,  loss  of  memory  for  recent  events,  and  incoherence  in 
conversation.  Sometimes  these  patients  are  childish,  casual 
and  irresponsible  in  word  and  deed,  and  exhibit  a  tendency  to 
make  silly  or  pointless  jests,  without  reference  to  the  conver- 
sation or  subject  under  discussion.  There  is  frequently  an 
alteration  in  character,  temperament,  and  tastes — facts 
which  can  only  be  ascertained  upon  information  supplied 
by  relatives  or  friends.  In  the  later  stages  delusions,  stupor, 
and  dementia  supervene. 

Irritative  symptoms.  The  following  varieties  of  seizure 
occur :  giddy  sensations,  '  petit  mal '  attacks,  generalised 
epileptic  convulsions,  and  fits  commencing  with  localised 
spasms.  The  last  are  of  importance,  and  may  be  subdivided 
into  two  types :  (1)  those  commencing  with  symptoms  of  a 
discharge  from  the  centres  in  the  post -frontal  region,  and 
(2)  those  commencing  with  symptoms  of  discharge  from  the 
motor  centres  in  the  precentral  convolution,  or  ascending 
frontal  gyrus. 

1.  In  the  first  type,  the  lit  commences  with  turning 
or  jerking  of  the  head  and  eyes  to  the  opposite  side.  These 
may  be  the  only  movements  which  occur,  consciousness  being 
preserved.  In  such  slight  attacks  the  patients  may  complain 
of  a  feeling  of  rotation  towards  the  side  of  the  lesion,  presum- 
ably due  to  the  apparent  displacement  of  the  environment 
consequent  on  the  rapid  involuntary  movements  of  the  eyes. 
On  the  other  hand,  a  fit  commencing  as  above  described  may 

16  * 


LM-I  nervous  diseases 

spread  to  the  face  and  mouth,  arm,  leg,  and  trunk,  or  it  may 
become  more  severe  and  generalised  with  loss  of  consciousness. 
It  is  important  to  note  that  these  attacks  are  never  ushered 
in  by  a  sensory  aura.  In  addition  to  the  character  of  the  fits, 
which  are  frequently  unobserved  or  incompletely  described, 
the  phenomena  of  the  post-convulsive  stage  may  be  of  great 
importance  in  the  localisation  of  the  disease.  After  severe 
seizures  the  head  and  eyes  are  in  paralytic  deviation  to  the 
side  of  the  lesion,  and  there  may  be  temporary  hemiparesis 
of  the  contralateral  limbs,  but  the  recovery  of  motor  power 
is  rapid.  The  movement  of  the  head  and  eyes  to  the 
opposite  side  is  the  last  to  recover.  Such  fits  point  to  the 
primary  irritation  and  secondary  exhaustion  of  the  centres  in 
the  post-frontal  region.  Temporary  hemianesthesia  of  the 
contralateral  side  may  be  present  after  a  severe  fit,  but  there 
is  never  hemianopsia.    The  loss  of  sensation  passes  off  quickly. 

2.  The  second  type  of  focal  fit  occurs  when  the  growth 
is  situated  in  the  left  frontal  region  and  the  patient  is  right- 
handed.  The  onset  is  characterised  by  a  sudden  loss  of 
speech,  followed  by  twitching  of  the  mouth  and  face  on  the 
opposite  side  (right),  and  often  accompanied  by  the  involun- 
tary emission  of  indefinite  articulate  sounds.  Consciousness 
may  remain  unimpaired  and  the  patient  signal  for  help, 
although  unable  to  speak.  The  discharge  may  spread  to  the 
neighbouring  centres,  and  a  Jacksonian  fit  with  the  ordinary 
march  supervene.  If  the  fit  be  severe  the  power  of  speech 
returns  slowly,  but  completely.  The  aphasia  is  purely  motor 
in  character,  the  patient  being  able  to  understand  what  is 
said  to  him.  The  association  of  such  attacks  with  mental 
symptoms  has  more  than  once  led  to  the  supposition  that  a 
patient  was  suffering  from  general  paralysis  of  the  insane ; 
but  in  contrast  to  what  occurs  in  that  disease,  recovery  from 
the  fit  is  more  rapid  and  the  after-effects  are  less  severe. 

The  occurrence  of  fits  with  focal  beginning  reveals  plainly 
the  side  of  the  tumour,  and  the  absence  of  a  sensory  aura 
makes  any  question  as  to  the  post-Rolandic  situation  of  the 
growth  untenable. 

Cranial  nerve  symptoms  are  usually  absent ;  but,  when 
present,  are  due  to  the  direct  pressure  of  the  growth.  They 
occur  upon  the  same  side  as  the  tumour,  and  indicate  that  the 
growth  is  situate  on,  or  extends  towards,  the  base  of  the  skull. 


DISEASES  OF  THE  BRAIN  245 

Those  chiefly  affected  are  the  olfactory  and  the  third  cranial 
nerves.  The  sixth  nerve  may  also  be  affected,  but  as  a  late 
and  indirect  symptom.  Olfactory  symptoms  are  especially 
important  from  the  point  of  localisation ;  and  when  other 
causes  may  be  excluded,  anosmia — especially  when  unilateral 
— is  of  especial  significance.  If  the  growth  is  basal  and  post- 
frontal,  it  may  press  directly  upon  the  optic  nerve,  resulting 
in  early  loss  of  sight  with  acute  optic  neuritis. 

The  motor  system.  Paresis  occurs  only  when  the  tumour 
impinges  on  the  motor  cortex  or  its  efferent  fibres,  in  which 
case  contralateral  hemiparesis  of  varying  degrees  may  result. 
The  most  definite  weakness  is  on  the  opposite  side  of  the  face, 
which  seems  to  be  represented  more  anteriorly  than  are 
the  limbs,  and,  as  in  all  cortical  lesions,  the  paresis  is  greater 
in  volitional  than  in  expressional  movement.  Apart  from 
such  signs,  which  are  not  strictly  of  frontal  origin,  is  the 
occurrence  of  tremor  on  the  side  homolateral  to  the  tumour. 
This  tremor  may  be  observed  in  both  the  upper  and  lower 
extremities,  but  is  more  constant  and  better  marked  in  the 
arm  than  in  the  leg.  In  character  the  tremor  is  fine,  rapid, 
and  vibratory,  and  may  often  be  more  easily  felt  than  seen. 
It  is  absent  during  muscular  rest,  and  can  be  best  demon- 
strated by  making  the  patient  extend  both  arms  horizontally 
in  front  of  him,  with  the  palms  directed  downwards  and  the 
fingers  extended.  It  will  then  be  noticed  that  the  homolateral 
arm  and  hand  are  in  a  state  of  constant  fine  vibratory  tremor, 
in  contrast  to  the  contralateral  arm,  in  which  such  tremor  is 
absent.  This  tremor  may  not  be  constantly  present  in  any 
given  case.  It  occurs  in  cases  in  which  there  is  no  evidence 
of  interference  with  the  motor  system — such  as  paresis,  as 
well  as  in  cases  where  such  paresis  exists.  In  a  few  cases 
such  tremor  was  observed  in  both  hands,  but  it  was  more 
regular  and  constant  and  of  the  typical  vibratory  nature  in 
that  homolateral  to  the  lesion. 

Sensory  system.  Affection  of  sensation  never  results 
directly  from  a  frontal  lesion,  and  only  occurs  as  a  temporary 
phenomenon  after  severe  fits,  when  the  discharge  has  presum- 
ably passed  across  the  motor  centres  to  the  post-Eolandic 
gyrus. 

Reflexes.  Deep  reflexes.  Tumours  limited  to  the  pre- 
frontal region  do  not  as  a  rule  cause  any  change  in  the  deep 


246  NERVOUS  DISEASES 

reflexes,  but  when  the  growth  extends  further  posteriorly  and 
impinges  on  the  motor  area,  the  contralateral  deep  reflexes 
are  increased.  When  there  is  a  considerable  increase  in 
the  intracranial  pressure,  the  deep  reflexes  are  increased  on 
both  sides. 

Superficial  reflexes.  The  examination  of  the  superficial 
abdominal  reflexes  is  of  the  greatest  importance,  as  they 
may  be  definitely  affected  in  cases  in  which  hemiparesis  or 
other  focal  signs  are  absent.  The  first  sign  is  absence  or 
diminution  of  the  epigastric  or  abdominal  reflexes  on  the  side 
opposite  to  the  tumour,  or  on  repeated  stimulation  the 
contralateral  reflexes  are  more  easily  exhausted  than  the 
homolateral.  This  alteration  of  the  superficial  abdominal  and 
epigastric  reflexes  is  not  a  specific  sign  of  frontal  lesions,  but 
merely  the  result  of  an  affection,  however  slight,  of  the 
pyramidal  system.  Its  value  lies  in  the  fact  that  it  is  the 
earliest  demonstrable  sign  of  a  commencing  hemiparesis. 

Still  more  important  as  positive  evidence  of  affection  of 
the  pyramidal  system,  is  the  presence  of  an  extensor  response 
on  stimulation  of  the  sole  of  the  foot.  In  cases  of  frontal 
tumour  the  contralateral  plantar  reflex,  when  not  extensor, 
may  be  of  an  indefinite  type  as  compared  with  that  of  the 
homolateral  side ;  when  this  is  associated  with  loss  or  diminu- 
tion of  the  corresponding  abdominal  reflexes,  its  significance 
is  that  of  a  typical  extensor  response. 

It  is  unnecessary  to  dwell  on  the  local  external  signs,  such 
as  localised  tenderness  on  pressure  over  the  skull,  protrusion 
of  the  eye,  or  bulging  of  the  cranium,  though  these  may  be 
of  value  in  diagnosis. 


TUMOURS  OF  THE  PRE-OENTRAL  REGION 
(MOTOR  AREA) 

The  pre-central  region  consists  of  the  ascending  frontal 
convolution  and  its  continuation  upon  the  mesial  surface. 
(Figs.  1,  2,  and  3.) 

The  general  symptoms  are  rarely  absent  when  the  tumour 
is  situated  in  this  locality  ;  but  as  this  region  contains  the 
motor  area  the  focal  symptoms  usually  occur  early  in  the 
course  of  the  disease  and  are  invariably  present. 


DISEASES  OF  THE  BRAIN 


247 


The  focal  symptoms  are  of  two  kinds — irritative  and 
paralytic. 

Irritative  signs.  Convulsive  seizures.  If  the  growth 
is  situated  on  or  near  the  surface,  convulsive  attacks  are 
frequent,  and  generally  have  a  local  commencement.  The 
site  of  the  tumour  in  the  face,  hand,  arm,  trunk  or  leg  areas 
on  one  side  of  the  brain  determines  the  onset  of  the  fit  in  the 


Fig.  77. — Shows  a  focal  or  Jacksonian  fit  involving  the  left  side 
of  the  face. 

muscles  of  the  face,  hand,  arm,  trunk  or  leg  respectively  of 
the  opposite  side  of  the  body.  These  seizures  are  motor  in 
character,  but  owing  to  the  proximity  of  the  sensory  cortical 
centres  behind  the  fissure  of  Eolando,  sensory  symptoms  not 
infrequently  coexist.     (Pig.  77.) 

A  fit  with  focal  commencement  may  result  in  a  generalised 
epileptic  seizure,  and  may  determine  the  onset  of  epileptic 
attacks.  A  localised  convulsion  usually  gives  rise  to  a  tem- 
porary mono-  or  hemiplegic  weakness  of  the  parts  convulsed. 


248  NERVOUS  DISEASES 

Paralytic  phenomena.  Progressive  monoplegia  or  hemi- 
plegia of  the  opposite  side  of  the  body  is  found  in  all  cases 
of  precentral  tumour.  This  may  be  observed  after  a  fit,  or 
series  of  fits,  and  although  at  first  transient  soon  becomes 
permanent  and  progressive.  This  is  especially  true  of  cases 
with  a  definite  and  constant  local  origin,  the  palsy  being 
earliest  observed  in  that  part  of  the  body  in  which  the  fit 


Fig.  78. — Showing  a  subcortical  tumour  into  which  a  hemorrhage 
has  taken  place. 

commences,  but  the  presence  of  such  paralyses  does  not 
necessarily  preclude  the  onset  of  further  fits  in  the  paralysed 
parts. 

If  the  tumour  is  subcortical,  convulsive  attacks  are  less 
common,  and  if  present,  are  general  in  character,  and  there  is 
a  greater  tendency  to  progressive  hemiplegia  with  spasticity. 
Rapid  wasting  of  the  hemiplegic  limbs,  which  however 
remain  spastic,  is  found  in  many  cases  of  subcortical  new 
growth. 

It  ought  to  be  borne  in  mind  that  a  sudden  onset  of  hemi- 


DISEASES  OF  THE  BRAIN  249 

plegia  does  not  negative  the  existence  of  a  cerebral  tumour, 
as  hemorrhage  into  the  tumour  or  surrounding  tissue  may 
occur.     (Fig.  78.) 

The  close  proximity  of  the  post-frontal  and  post-central 
regions  to  the  motor  area  proper  (precentral)  renders  them 
liable  to  suffer,  so  that  psychical  and  sensory  changes  are  not 
uncommon.     The  fits  are  often  associated  with  numbness  or 


Fig.  79. — Showing  a  subcortical  tumour  involving  the  centrum  ovale, 
and  lenticular  nucleus  on  the  left  side.  There  is  considerable 
distension  of  the  lateral  ventricles,  and  the  brain  is  pushed  over 
towards  the  right  side. 

tingling  in  the  parts  convulsed,  and  temporary  hemianesthesia 
may  coexist.  In  subcortical  tumours  permanent  loss  of 
sensation  is  frequent,  owing  to  the  propinquity  of  the  motor 
and  sensory  paths,  but  hemianopsia  is  not  present. 

Motor  aphasia  of  varying  degrees  may  be  observed  when  the 
tumour  is  situated  in  the  left  hemisphere :  when  the  mental 
condition  appears  to  be  more  affected  than  really  is  the  case. 


250  NERVOUS  DISEASES 

Reflexes.  On  the  paralysed  side  the  tendon  jerks  are 
increased,  the  plantar  is  of  the  extensor  type,  and  the  super- 
ficial  abdominal  reflexes  are  absent. 

TUMOURS  OF  THE  POST-CENTRAL  AND  PARIETAL 
REGIONS   (SENSORY  AREA) 

This  region  embraces  the  ascending  and  superior  parietal 
convolutions.  The  separation  of  the  post-central  convolution 
into  a  primary  sensory  portion  lying  in  and  immediately 
behind  the  fissure  of  Rolando,  and  a  '  psycho-sensory '  in 
close  relation  with  the  superior  parietal  lobule,  has  been 
already  mentioned. 

The  post-central  area  has  immediately  in  front  of  it,  just 
across  the  fissure  of  Rolando,  the  pre-eentral  or  motor  area. 
Posteriorly  are  the  higher  visual  centres  in  the  angular  gyrus, 
and  inferiorly,  within  and  across  the  fissure  of  Sylvius,  is  the 
auditory  centre  in  the  superior  temporal  convolution. 

It  is  therefore  obvious  that  the  extension  of  a  tumour 
growth  from  the  parietial  region  in  a  direction  either  forwards, 
downwards,  or  backwards  will  give  rise  to  symptoms  corre- 
sponding to  the  invaded  area. 

In  man  clinical  evidence  points  to  the  post-central  gyrus 
as  the  cortical  area  for  the  primary  recognition  of  the  senses 
of  active  and  passive  movement,  of  tactile  localisation  and 
discrimination,  and  possibly  to  a  less  extent  of  painful  and 
thermal  sensibility. 

The  representation  of  the  sensibilities  of  active  and  passive 
movement  and  tactile  localisation  would  further  appear  to  be 
situated  posterior  to  the  corresponding  motor  areas  and  in 
close  connexion  with  them. 

The  examination  of  cases  in  which  there  have  been  cortical 
lesions  situated  in  the  post-central  gyrus  and  parietal  region 
has  demonstrated  the  following  facts  as  regards  the  quality 
and  distribution  of  the  sensory  loss :  (1)  Tactile  localisation 
and  the  sense  of  movement  are  most  impaired,  even  to 
complete  abolition.  Painful  and  thermal  sensibility  may 
not  be  affected  at  all ;  when  affected  there  is  not  complete 
loss.  A  relative  diminution  of  deep  and  painful  pressure 
sensibility  may  also  be  present. 

2.  The    sensory   loss    is   more    pronounced   in   the   distal 


DISEASES  OF  THE  BRAIN  251 

than  in  the  proximal  parts  of  the  limb.  The  affected  sl<in 
areas  have  no  relation  to  a  peripheral  or  spinal  sensory  distri- 
bution, but  appear  to  be  sensory  analogues  of  the  motor 
disability  following  upon  lesions  of  the  corresponding  areas 
the  pre-central  gyrus.  The  extent  of  the  sensory  loss  may  be 
restricted  to  more  or  less  definite  areas — such  as  the  hand, 
a  limb,  or  one  side  of  the  body.  Where  such  sensory  loss 
is  well  defined,  all  forms  of  sensibility  are  more  or  less 
affected. 

According  to  Mills,1  the  stereognostic  sense,  or  sense  of 
recognition  of  the  size,  shape,  and  consistency  of  objects 
placed  in  the  hand,  is  situated  in  the  posterior  part  of  the 
superior  parietal  lobule.. 

The  idea  of  a  stereognostic  centre  presumes  an  intact 
condition  of  the  cutaneous  and  muscular  sensibilities.  The 
occurrence  of  cases,  therefore,  in  which  astereognosis  is  found 
without  any  other  objective  sensory  loss,  would  favour  the 
existence  of  such  a  centre.  We  have,  however,  been  unable 
to  satisfy  ourselves,  either  by  personal  observation  or  from 
a  study  of  the  literature,  that  astereognosis  exists  without 
some  defect  of  the  primary  sensibilities. 

The  general  symptoms  of  intracranial  tumour  are  present. 

The  focal  symptoms  of  a  tumour,  strictly  limited  to  this 
region,  consist  of  loss  or  impairment  of  cutaneous  sensibility 
and  of  astereognosis  on  the  opposite  side  of  the  body. 
Cutaneous  sensibility  is  rarely  completely  lost,  but  tactile 
sensibility — especially  that  to  cotton  wool — is  impaired  to 
the  greatest  extent.  The  senses  of  active  movement  and  of 
passive  position  of  the  limbs  are  also  impaired  by  lesions  in 
this  locality. 

Irritative  symptoms.  These  consist  of  a  sensation  of 
numbness  and  of  tingling  commencing  locally  in  some  portion 
of  the  body,  but  spreading  so  as  to  become  general  over  the 
whole  of  the  opposite  side.  It  is  rare  for  a  seizure  com- 
mencing with  a  local  sensory  aura  to  remain  purely  sensory, 
for  the  cortical  discharge  spreads  to  the  corresponding  motor 
area,  and  Jacksonian  motor  attacks  follow  immediately  in  the 
wake  of  the  subjective  sensation. 

These  phenomena  were  observed  in  three  cases.  In  one, 
in  which  a  glioma  was   limited   to   the   post-central   gyrus 

Mills,  Univ.  of  Pennsylvania  Med.  Bulletin,  1906. 


252  NERVOUS  DISEASES 

opposite  the  hand  and  face  areas,  the  fit  commenced  with  a 
subjective  sensation  of  numbness,  tingling,  and  '  drawing '  of 
the  face  and  lingers  on  the  opposite  side.  Consciousness  was 
retained,  and,  in  answer  to  questions,  the  sensation  was 
described  as  having  reached  the  elbow  thirty-five  seconds 
from  the  commencement  of  the  attack.  The  motor  convulsion 
then  started  in  the  face  and  hand,  and  rapidly  spread  to  the 
arm.  trunk,  and  leg,  and  later  became  generalised,  with  loss 
of  consciousness.  On  recovery  the  patient  stated  that  the 
subjective  sensation  had  reached  the  shoulder  before  the  motor 
convulsion  in  the  face,  arm,  and  forearm  had  commenced. 
After  the  seizure,  partial  hemiplegia — chiefly  in  the  hand  and 
face — with  complete  hemianesthesia  to  all  forms  of  sensation, 
was  detected.  The  deep  reflexes  were  exaggerated,  the 
abdominal  reflexes  were  absent,  and  the  plantar  response 
was  indefinitely  extensor.  Eventually  there  was  complete 
return  of  motor  power,  and  sensation  became  normal,  except 
in  the  hand. 

In  two  cases,  in  which  a  tumour  was  found  in  the  ascending 
parietal  gyrus — opposite  respectively  the  leg  and  trunk  areas 
of  the  motor  zone — the  fits  were  at  first  characterised  by  a 
sensation  of  movement  in  the  foot  and  abdomen  upon  the 
opposite  side,  although  no  obvious  movement  was  detected. 
Later  on,  in  both  cases,  motor  Jacksonian  seizures  appeared, 
commencing  in  those  portions  of  the  body  to  which  the 
subjective  sensations  were  referred. 

Paralytic  symptoms.  These  consist  of  loss  or  impairment 
of  sensation  upon  the  opposite  side  of  the  body,  more 
particularly  of  tactile  sensibility  and  the  sense  of  passive 
position.  Frequently  vaso-motor  disturbances,  consisting  of 
either  elevation  or  depression  of  the  surface  temperature, 
are  observed. 

Motor  system.  Except  in  the  early  stages,  motor  weak- 
ness of  the  opposite  limbs  is  present.  Later  on  this  may 
become  considerable,  and  is  not  infrequently  attended  by 
some  degree  of  ataxia.  A  further  feature  is  the  tendency  to 
a  somewhat  pronounced  wasting  of  the  muscles  of  the  paralysed 
limbs  with  rigidity,  but  occasionally  with  flaccidity.  In  the 
latter  t}Tpe,  an  increased  myotatic  irritability  is  observed. 

Beflexes.  Loss  or  diminution  of  the  superficial  reflexes 
is  almost  constant  on  the  opposite  side.     The  deep  reflexes 


DISEASES  OF  THE  BRAIN  253 

vary ;  in  our  experience  they  have  always  been  increased  on 
the  opposite  side,  but  some  observers  (Mills  and  others)  have 
described  their  diminution  or  absence. 

TUMOURS  OF  THE  OCCIPITAL  LOBE 

The  general  symptoms  of  intracranial  tumour  are  usually 
present.  Headache  is  occipital  in  position  and  optic  neuritis 
is  often  intense. 

The  focal  symptoms  of  a  tumour  of  the  occipital  region 
are,  subjective  and  objective  disturbances  of  vision  in  the 
homonymous  fields  opposite  the  lesion. 

The  subjective  symptoms  consist  of  visual  sensations, 
usually  of  light  or  of  colours,  sometimes  accompanied  by  a 
feeling  of  numbness  or  paresthesia  in  the  limbs  on  the  side 
of  the  body  opposite  to  the  lesion.  The  visual  hallucinations 
usually  have  a  hemianopic  distribution.  A  combination  of 
subjective  visual  symptoms  and  numbness  may  be  mistaken 
for  migraine. 

Sooner  or  later,  however,  loss  of  vision  of  a  definitely 
hemianopic  type  occurs.  The  subjective  sensations  may 
persist  even  after  the  development  of  the  objective  loss,  but 
tend  gradually  to  disappear.  Although  the  defect  of  vision 
is  usually  hemianopic  involving  the  half-fields,  the  loss  is 
sometimes  limited  to  one  or  other  visual  quadrant ;  but  this 
is,  perhaps,  less  frequent  in  tumours  than  in  vascular  lesions. 

Although  unaware  of  the  hemianopic  nature  of  the  visual 
defect,  the  patient  may  early  complain  of  loss  of  sight.  On 
examination  of  the  fundus  oculi,  optic  neuritis  may  be 
observed.  It  is,  therefore,  important  to  test  the  visual  fields, 
as  it  is  easy  to  attribute  the  defect  of  sight  to  the  influence 
of  the  local  neuritic  condition. 

If  the  tumour  is  situated  on  the  mesial  aspect  of  the 
occipital  lobe,  homonymous  hemianopsia  to  the  opposite  side 
is  present.  This  is  due  to  destruction  of  the  primary  visual 
centre  situated  in  the  lips  of  the  calcarine  fissure.  Irritative 
symptoms,  when  present,  are  crude — such  as  flashes  or 
sparks  of  light. 

In  some  cases,  where  the  growth  is  situated  well  forwards 
and  encroaches  upon  the  under  surface  of  the  occipital  lobe, 
cerebellar  symptoms  may  be  present  from  direct  pressure  upon 
the    cerebellum.      The    '  symptom-complex '    in    these    cases 


254  NERVOUS  DISEASES 

consists  of  homonymous  hemianopsia,  hemianesthesia,  and 
slight  motor  weakness  on  the  contralateral  side,  in  association 
with  ataxia,  hypotonia,  and  nystagmus,  slower  and  more 
marked  towards  the  side  of  lesion  on  lateral  conjugate 
movements  on  the  homolateral  side.  These  last  symptoms 
arise  from  pressure  upon  the  lateral  lobe  of  the  cerebellum. 

If  the  tumour  is  situated  on  the  outer  aspect  of  the 
occipital  lobe,  subjective  symptoms  of  a  more  specialised 
t}rpe  usually  occur  early ;  while  the  extension  of  the  growth 
forwards  along  the  convexity  results  in  a  mind-blindness, 
and  word-blindness  from  involvement  of  the  higher  visual 
centres  in  the  angular  gyrus. 

Hemianesthesia  and  hemiplegia  are  also  produced  by  a 
forward  and  inward  extension  of  a  tumour  situated  sub- 
cortically  in  the  occipital  lobe.  These  signs  may  be  present 
without  any  subjective  visual  sensations. 

Convulsive  seizures  may  also  occur  in  cases  of  tumour 
of  the  occipital  region.  These  are  either  general  or  limited 
to  the  limbs  on  the  opposite  side,  in  consequence  of  an 
increase  of  the  intracranial  pressure.  Localised  convulsions, 
preceded  by  a  sensory  warning,  may  also  occur. 

The  pupillary  light  reflex  is  unimpaired  in  cases  of  tumour 
limited  to  the  occipital  cortex  or  subcortical  tissues. 

Reflexes.  As  in  all  cerebral  tumours,  a  loss  or  diminu- 
tion of  the  superficial  reflexes  on  the  opposite  side  may  be 
one  of  the  earliest  signs,  even  when  hemianopsia  is  the  only 
physical  sign.  In  the  later  stages,  with  the  occurrence  of 
obvious  motor  symptoms,  the  deep  reflexes  are  increased  and 
a  plantar  extensor  response  appears  upon  the  opposite  side. 

TUMOURS  OF  THE  TEMPOBO-SPHENOIDAL  LOBE 

For  purposes  of  localisation  this  lobe  is  divided  into  two 
portions  :  (a)  the  tip  of  the  lobe  upon  the  mesial  aspect  of  the 
brain— the  uncinate  gyrus  ;  and  {b)  the  superior  temporal 
gyrus  upon  the  convexit}7. 

The  general  symptoms  of  intracranial  tumour  are  present 
in  most  cases. 

The  focal  symptoms  are  separately  described,  according  as 
the  lesion  is  situated  in  the  uncinate  region,  or  in  the  superior 
temporal  gyrus. 


DISEASES  OF  THE  BRAIN  255 


i.   The  uncinate  gyrus 

A  tumour  situated  in  this  locality  reveals  itself  by  the  onset 
of  seizures  having  a  warning  of  a  subjective  sensation  of  smell 
or  flavour.  The  sensations  are  sometimes  of  an  unpleasant 
or  perverted  character,  and  may  be  so  real  to  the  patient  that 
he  dreads  associating  with  others.  The  attacks  are  charac- 
terised by  the  crude  warning  sensation  just  described,  which 
may  or  may  not  be  associated  with  a  '  dreamy  state.'  The 
dreamy  state  maybe  of  the  nature  of  a  'reminiscence' — as 
if  that  which  is  happening  has  been  previously  experienced  ; 
or  of  a  feeling  of  unreality — as  if  surrounding  objects  were 
unfamiliar.  An  intense  feeling  of  fear  or  of  impending  death 
may  be  associated  with  it.  During  the  seizure  smacking  move- 
ments of  the  lips,  chewing  movements  of  the  jaw,  and  sometimes 
spitting  have  been  observed.  Occasionally  when  the  attack  is 
over,  the  patient  has  a  subjective  sensation  of  intense  hunger, 
or  a  desire  to  go  to  stool.  During  the  unconscious  stage,  the 
arm  on  the  side  opposite  the  lesion  may  be  moved  about  in  a 
slow,  deliberate,  and  apparently  purposive  manner. 

These  attacks  may  be  succeeded  by  transient,  bilateral, 
incomplete  loss  of  the  senses  of  taste  and  smell.  In  cases 
with  destructive  lesions  of  the  uncinate  lobe,  there  is  never 
complete  loss  of  taste  or  smell,  but  a  bilateral  impairment  is 
not  uncommon. 

2.   Superior  temporal  gyrus 

In  the  early  stages  or  in  localised  lesions,  subjective  sensa- 
tions of  sound  are  heard  by  the  patient.  When  the  tumour 
is  more  extensive,  convulsive  movements — often  limited  to  the 
face  and  arm  of  the  opposite  side,  but  sometimes  becoming 
unilateral  with  loss  of  consciousness — may  occur,  and  are  due 
to  irritation  of  neighbouring  centres ;  generalised  epileptic 
seizures  are  also  sometimes  present.  Incomplete  cortical 
deafness  of  the  opposite  ear  may  also  be  observed,  both  after 
the  seizures  and  as  an  independent  focal  symptom. 

In  right-handed  persons  a  focal  lesion  of  the  left  superior 
temporal  gyrus  causes  not  only  cortical  deafness  in  the 
opposite  ear,  but  also  word-deafness.  A  point  of  importance 
in  differential  diagnosis  is   the  fact  that,  in  abscess  of  the 


256  NERVOUS  DISEASES 

temporo-sphenoidal  lobe  arising  from  unilateral  suppurative 
middle  ear  disease,  deafness  due  to  the  cortical  lesion  is  in 
the  ear  opposite  that  which  is  diseased. 

The  cranial  nerves  are  not  usually  affected. 

Motor  system.  Fits  when  present  commence  in  the  face 
or  arm.  In  the  early  stages  paresis  of  the  face  or  arm  may 
be  observed,  which  progresses  to  hemiplegia,  if  the  tumour  is 
of  considerable  size. 

Reflexes.  One  of  the  earliest  signs  of  tumour  in  this 
region  is  impairment  or  loss  of  the  superficial  abdominal 
reflexes  on  the  opposite  side.  The  deep  reflexes  may  be  in- 
creased, but  have  been  found  to  be  diminished  in  many  cases, 
and  a  plantar  extensor  response  develops  in  the  later  stages. 

Sphincters.  Sphincter  control  over  the  bladder  and  rectum 
may  be  impaired  from  lack  of  voluntary  control,  owing  to  the 
mental  condition. 

TUMOURS  OF  THE  MIDBRAIN  AND  SUBTHALAMIC 

REGION 

In  addition  to  the  general  symptoms  of  intracranial  tumour, 
more  or  less  definite  localising  signs  may  be  observed.  When 
present  they  are  found  upon  the  same  side  as  the  lesion. 
They  are  : — 

1.  Weakness  of  the  upward  movement  of  the  eyeballs. 

2.  Defective  pupillary  light  reaction. 

3.  Ectopia  or  eccentric  position  of  the  pupil. 

4.  Paresis  or  paralysis  of  the  third  cranial  nerve. 

5.  Defective  sensibility  over  the  fifth  cranial  nerve  area. 
Hemiplegic     weakness,     accompanied    by    more    or    less 

rhythmical  tremor  and  ataxia,  may  be  found  in  the  limbs  on 
the  side  opposite  the  lesion.  Sometimes  an  associated  hemi- 
anaesthesia  is  also  observed.  Occasionally  these  signs  are 
bilateral.  Should  hemiataxia  and  tremor  be  present  in  the 
limbs  upon  the  same  side  as  the  tumour,  they  are  due  to  an 
associated  involvement  of  the  cerebellar  and  rubro-spinal 
systems. 

Various  forms  of  seizure  may  be  associated  with  tumours 
in  this  locality.  Sometimes  generalised  convulsive  attacks 
are  observed,  at  other  times  impairment  of  consciousness, 
which  may  deepen  into  coma,  and  be  accompanied  by  transient 
residual  third-nerve  paralysis. 


DISEASES  OF  THE  BRAIN 


.'57 


TUMOURS  OF  THE  LATERAL  VENTRICLE 

The  onset  of  the  general  symptoms,  which  are  intense,  is 
rapid.  Definite  local  symptoms  are  often  absent  or  delayed; 
but  in  all  cases  the  supra-tentorial  situation  of  the  growth  is 
made  clear  by  the  early  diminution  of  mental  acuteness,  often 


Fig.  80. — Shows  a  tumour  of  the  choroid  plexus  involving  the 
left  lateral  ventricle. 

associated  with  psychical  symptoms  resembling  those  seen 
in  cases  of  tumour  of  the  frontal  lobe.  In  addition,  slight 
bilateral  spasticity — sometimes  more  marked  on  the  side 
opposite  the  tumour — an  increase  of  the  deep  reflexes,  dimi- 
nution of  the  superficial  reflexes,  with  either  flexor  or  extensor 
plantar  response,  are  observed.  When  an  extensor  plantar 
response  is  present,  some  degree  of  motor  weakness  of  a 
spastic  type  will  be  detected.  In  the  later  stages,  false 
localising  signs  arise  from  the  increasing  pressure  in  the 
mfra-tentorial  chamber.     These  are  :  giddiness,  unsteadiness, 

17 


258  NERVOUS  DISEASES 

and  general  ataxia,  sometimes  associated  with  nystagmus 
and  paresis  of  the  sixth  nerve.  In  the  terminal  stages 
symptoms  of  bulbar  anfemia,  characterised  by  respiratory  and 
vaso-motor  paralysis,  foretell  the  impending  death.    (Fig.  80.) 

TUMOURS  OF  THE  THIRD  VENTRICLE 

The  general  symptoms  may  be  of  early  or  late  onset 
The  cerebral  symptoms  are  more  or  less  general  and  bilateral 
in    character  and  consist   of  mental   impairment,   bilateral 


Fig.  80a. — Illustrates  a  tumour  of  the  third  ventricle,  spreading  down- 
wards along  the  '  iter  '  and  appearing  in  the  fourth  ventricle. 

spasticity  with  some  motor  weakness,  increase  of  the  deep 
reflexes,  diminution  of  the  superficial  reflexes  and  extensor 
plantar  responses. 

The  only  localising  signs  present  in  these  cases  are:  (1) 
the  early  impairment  of  the  reaction  of  the  pupils  to  light ; 
(2)  the  associated  weakness  of  the  upward  movements  of 
the  eyes;  (3)  the  occasional  occurrence  of  ectopia  pupillae ; 
(4)  the  rapid  failure  of  vision  at  a  time  when  the  appearance 
of  the  fundus  oculi  indicates  an  acute  stage  of  optic  neuritis ; 
or  (5)  early  failure  of  vision  without  optic  neuritis. 


DISEASES  OF  THE   BRAIN 


259 


The  first  three  symptoms  are  due  to  pressure  upon 
the  third  nerve  nucleus ;  the  last,  to  pressure  upon  the 
optic  chaisma  by  the  anterior  end  of  the  distended  third 
ventricle.     (Fig.  80a.) 

TUMOURS  OF  THE  FALX  CEREBRI   (INCLUDING 
THE  MESIAL  ASPECT  OF  THE  CEREBRUM) 

Tumours  in  this  locality  are  of  slow  growth,  and  are 
either   endotheliomatous   or  fibro-sarcomatous   in  character, 


.4^ 


Fig.  81. — Shows  a  localised,  circumscribed  tumour  involving  the  precuneus. 

and  tend  to  displace  rather  than  infiltrate  the  cerebral 
substance. 

The  general  symptoms  are  of  slow  development ;  but 
headache  commences  well  in  advance  of  vomiting  and  optic 
neuritis.  The  headache  is  commonly  vertical  in  position  and 
attended  by  pain  on  pressure. 

The  focal  symptoms  are  early  and  are  at  first  of  an 
irritative  character.  The  nature  of  the  seizures  depends 
upon  the  situation  of  the  growth.  If  this  is  situated  in  the 
anterior  half  of  the  falx,  bilateral  and  general  epileptic  attacks 
are  present,  sometimes  on  one  side  and  sometimes  on  the 
other.  These  may  be  accompanied  by  an  early  development 
of  mental  failure.     The  clinical  picture  is  that  of  a  bilateral 

17* 


260  NERVOUS  DISEASES 

frontal  lesion,  and  may  bo  readily  mistaken  for  general 
paralysis  of  the  insane. 

If  the  tumour  grows  from  the  posterior  half  of  the  falx 
cerebri,  the  irritative  focal  symptoms  consist  of  Jacksonian 
attacks,  commencing  simultaneously  in  both  lower  extremities, 
or  they  may  commence  first  in  one  and  then  involve  the  other 
leg,  or  begin  alternately  in  one  or  other  foot. 

The  recurrence  of  such  attacks  is  followed  by  a  gradual 
and  progressive  paralysis  of  both  lower  limbs.  It  is  rare  for 
the  motor  system  to  be  alone  affected,  sensory  phenomena, 
both  irritative  and  paralytic,  occurring  simultaneously. 

The  deep  reflexes  are  increased  on  both  sides,  especially 
in  the  lower  limbs,  along  with  double  extensor  response  and 
absence  of  the  superficial  abdominal  reflexes. 

In  rare  cases  visual  phenomena  in  the  form  of  subjective 
flashes  of  light  have  been  observed,  but  changes  in  the  visual 
fields  have  never  been  definitely  detected.  In  one  case  in 
which  these  symptoms  were  present,  the  mental  condition 
and  a  well-marked  degree  of  secondary  optic  atrophy  pre- 
cluded any  trustworthy  objective  investigation. 

Occasionally  the  growth  extends  entirely  into  one  hemi- 
sphere, in  which  event,  although  the  irritative  symptoms 
may  be  bilateral,  the  paralytic  phenomena  are  predominately 
on  one  side. 


TUMOURS  OF  THE  CORPUS  CALLOSUM 

The  general  symptoms  are  early  and  well  defined ;  optic 
neuritis  may  be  intense  and  mental  symptoms  pronounced. 

A  rapid  loss  of  vision  takes  place  in  cases  where  the 
anterior  portion  and  the  knee  of  the  corpus  callosum  are 
affected.  This  is  a  striking  feature  and  a  point  of  diagnostic 
importance,  as  the  well-marked  optis  neuritis  associated  with 
blindness,  but  without  atrophic  changes  in  the  disc,  is  quite 
unusual,  and  is  to  be  accounted  for  by  direct  pressure  of 
the  growth  upon  the  optic  tracts. 

General  epileptic  seizures  are  not  uncommon,  and  paralysis 
of  one  or  other,  or  both,  sides  of  the  body  develops  as  the 
tumour  infiltrates  the  centrum  ovale.  A  particular  feature 
observed  on  the  hemiplegic  side  is  the  difficulty  which  the 
patient  experiences  in   relaxing   his   grasp  from  any  object 


DISEASES  OF  THE  BRAIN  261 

which  he  may  have  in  his  hand.  Apraxia,  or  the  inability  to 
use  an  object  for  its  proper  purpose — although  its  name  and 
the  purpose  for  which  it  is  used  are  known — is  sometimes 
found,  and  when  present  may  be  regarded  as  a  diagnostic 
sign  of  importance  in  lesions  of  this  structure. 

TUMOURS  OF  THE  PITUITARY  BODY 

Tumours  of  the  pituitary  body  may  or  may  not  be  asso- 
ciated with  acromegaly,  and  may  or  may  not  be  characterised 
by  the  signs  of  intracranial  tumour.  Two  types  may  be 
described. 

In  the  first  type  there  is  a  bilateral  temporal  hemianopsia 
with  pallor  of  the  inner  (nasal)  half  of  the  optic  discs,  without 
evidence  of  optic  neuritis.  The  general  symptoms  may  be 
absent,  but  some  degree  of  mental  impairment  is  usually 
present — chiefly  in  the  direction  of  loss  of  memory  and  slow 
cerebral  action.  The  motor,  sensory,  and  reflex  systems 
show  no  change. 

A  second  type  is  found  in  which  the  general  symptoms  of 
intracranial  tumour  are  well  marked ;  frontal  headache,  optic 
neuritis,  and  mental  impairment  being  early  and  prominent 
features.  In  these  cases  acromegaly  never  develops,  and  the 
loss  of  vision  is  rapid.  If  the  case  is  seen  in  the  early  stages, 
bitemporal  hemianopsia  may  be  observed;  but  this  quickly 
gives  place  to  blindness,  affecting  one  side  in  advance  of 
the  other,  so  that  temporal  hemianopsia  is  found  on  one  side 
and  loss  of  vision  on  the  other.  This  stage  soon  passes  into 
that  of  complete  blindness,  and  if  now  examined,  the  degree 
of  blindness  is  out  of  all  proportion  to  the  changes  in  the 
fundus  of  the  eye,  little  or  no  atrophy  being  detected.  The 
mental  symptoms  are  obtrusive — inattention,  loss  of  memory, 
childish  behaviour,  and  rapidly  developing  dementia. 

In  addition  to  the  visual  defects,  interference  with  the 
functions  of  the  third  and  sixth  nerves  on  one  or  both  sides  is 
frequently  observed.  Occasionally  pain  over  the  ophthalmic 
branch  of  the  fifth  nerve  is  described.  The  other  cranial 
nerves  are  not  affected,  except  loss  of  smell,  which  may  be 
bilateral  and  complete. 

Generalised  epileptic  fits,  muscular  lrypertonicity,  and 
slight  spasticity  of  the  limbs   without  obvious  paresis,   are 


262  NERVOUS  DISEASES 

occasional  symptoms.  Sensation  is  not  affected.  The  deep 
reflexes  are  exaggerated,  the  superficial  are  diminished  along 
with  flexor  or  indefinite  plantar  responses. 

Sphincter  action  is  not  impaired,  but,  owing  to  the  mental 
condition,  there  is  a  want  of  control  over  the  evacuations. 


TUMOURS  OF  THE  CEREBELLUM 

The  general  symptoms  of  intracranial  new  growth  are  well- 
marked  and  early  features.  Headache  is  a  constant  and  early 
symptom,  being  as  a  rule  most  intense  and  persistent  in  the 
occipital  region.  In  a  few  cases  it  is  frontal  in  position  ;  but  it 
is  not  uncommon  for  complaint  to  be  made  of  pain  radiating 
down  the  back  of  the  neck.  It  may  be  associated  with  attacks 
of  vomiting.  Optic  neuritis  is  an  invariable  accompaniment 
of  cerebellar  tumour.  It  is  an  early  sign,  intense  in  character, 
and  often  quite  out  of  proportion  to  the  localising  symptoms. 
Its  onset  is  acute,  vision  is  impaired  early — either  temporarily 
or  permanently — from  consecutive  atrophic  changes  in  the 
optic  nerve.  In  character  the  neuritis  is  similar  to  that 
seen  in  other  intracranial  tumours ;  but  in  some  cases  the 
appearance  of  the  fundus  resembles  that  seen  in  albuminuric 
retinitis.  Vertigo  or  giddiness  is  another  characteristic 
symptom,  and  may  consist  merely  of  an  indefinite  sensation 
of  unsteadiness,  or  of  a  definite  subjective  sense  of  movement 
towards  the  sound  side.  External  objects  also  appear  to 
move  in  the  same  direction. 

Cranial  nerves.  Symptoms  referred  to  the  cranial  nerves 
are  chiefly  ocular  in  character,  and  consist  of  slight  paresis 
of  the  external  rectus  muscle  on  the  side  of  the  lesion.  This 
may  occasionally  give  rise  to  diplopia,  which  is  as  a  rule 
transitory,  and  unaccompanied  by  obvious  strabismus. 
A  slighter  degree  of  paresis  of  the  opposite  external  rectus 
may  sometimes  be  present.  Occasionally  some  weakness 
of  conjugate  movement  towards  the  side  of  the  lesion — 
particularly  a  want  of  sustained  movement — may  be  observed. 
In  cases  with  optic  atrophy,  the  eyes  when  at  rest  are 
usually  deviated  towards  the  sound  side. 

In  some  cases,  after  acute  attacks  of  vomiting  and  headache, 
the  eye  on  the  side  of  the  lesion  may  be  directed  downwards 


DISEASES  OF  THE  BRAIN 


263 


and  inwards,  and  the  others  upwards  and  outwards.  This 
is  known  as  '  skew  deviation,'  and  is  probably  an  irritative 
rather  than  a  paralytic  symptom.     (Fig.  82.) 

Nystagmus  is  always  present  and  is  a  valuable  localising 
sign.  In  typical  cases,  it  consists  of  slow  and  deliberate 
jerkings  of  both  eyes  on  lateral  conjugate  movement  to  the 
side  of  the  lesion ;  these  are  in  marked  contrast  to  the  finer 
and  more  rapid  movements,  which  take  place  on  conjugate 
deviation  to  the  opposite  side.  Nystagmus  may  also  be 
present,  but  less  well  marked  on  upward  and  downward 
movements  of  the  globes,  and 
consists  of  rotatory  upward  and 
downward  movement  to  the  side 
of  the  lesion. 

The  pupils  may  be  dilated,  and 
in  cases  with  pronounced  optic 
atrophy  the  light-reflex  is  lost. 

In  rare  cases  of  tumour  of 
the  lateral  lobe  the  sensory 
division  of  the  fifth  nerve  may 
be  implicated.  The  anaesthesia 
is  incomplete,  and  the  motor 
root  is  never  involved. 

Occasional  and  slight  weak- 
ness of  the  facial  muscles  on  the 
side  of  the  lesion  may  be  noticed. 

Subjective  auditory  symptoms  are  rarely  present,  and  an 
affection  of  hearing  is  no  part  of  the  cerebellar  '  syndrome.' 

The  other  cranial  nerves  are  not  affected. 

Motor  symptoms.  Paresis  of  slight  degree,  but  of  definite 
character,  is  present  in  the  limbs  on  the  side  of  the  lesion. 
This  weakness  is  most  marked  in  acute  cases  and  is  best  seen 
in  the  muscles  of  the  back.  A  defective  muscular  tone 
(atonia)  is  associated  with  the  muscular  paresis  of  the 
homolateral  side.  The  limbs  when  handled  are  found  to  be 
unusually  limp  and  flaccid,  and  the  muscles  are  soft  and 
flabby.  Atonia  of  this  type  may  coexist  with  increased 
tendon  reflexes — a  point  which  is  in  striking  contrast  to 
the  atonia  seen  in  tabes  dorsalis. 

Ataxia  is  more  or  less  characteristic.  It  is  present  in  the 
homolateral  musculature,  although  that  on  the  opposite  side 


Fig.  82. — Showing  '  skew  de- 
viation '  from  lesion  of  the 
cerebellum. 


L>G4 


NERVOUS  DISEASES 


may  be  slightly  affected.  It  is  due  to  a  lack  of  accurate 
co-operation  and  association  of  the  synergic  muscles  effecting 
muscular  movements,  and  not  to  the  impairment  of  the 
muscular  sense  or  sense  of  position  of  the  limbs,  as  occurs  in 
tabes,  and  it  is  not  increased  on  closure  of  the  eyes.  Hence  it 
is  only  present  during  the  performance  of  a  movement.  In 
the  'finger-nose  test,'  for  example,  the  ataxic  deviation  of  the 
finger  is  marked  at  the  outset,  but  becomes  less  pronounced 
towards  the  completion  of  the  act,  and  finally  ceases.  The 
ataxia  is  most  marked  in  the  trunk  and  lower  limbs. 

Attitude.  Considerable 
attention  has  been  devoted 
to  the  position  of  the  head 
in  cerebellar  disease. 
When  standing  or  sitting 
the  head  is  generally  tilted 
so  that  the  occiput  ap- 
proximates to  the  shoulder 
on  the  side  of  the  lesion, 
and  the  chin  is  deviated 
to  the  opposite  side.  But 
the  converse  position  has 
also  been  described.  When 
standing,  the  homolateral 
leg  is  slightly  everted  and 
abducted,  so  as  to  broaden 
the  supporting  base ;  the 
homolateral  shoulder  is  held  higher  than  its  fellow,  and 
there  is  definite  lordosis. 

The  gait  of  cerebellar  disease  is  described  as  reeling, 
staggering,  or  drunken.  There  is  a  tendency  both  to  stumble 
and  fall,  as  well  as  to  deviate  to  the  side  of  the  lesion.  The 
patient  is  fully  conscious  of  this  deviation,  and  endeavours  to 
overcome  it  by  rotation  of  the  body  towards  the  sound  side. 

There  is  a  greater  tendency  to  fall  to  the  side  of  the  lesion 
than  to  the  opposite  side,  and  if  left  alone  the  patient  would 
tend  towards  a  '  circus  movement '  to  the  side  of  the  lesion. 
This,  however,  is  corrected  automatically  by  rotation  of  the 
whole  body  towards  the  sound  side,  which  if  overdone  leads 
to  an  appearance  of  staggering  and  deviation  towards  the 
sound  side.     In  any  doubtful  case  it  is  advisable  to  test  the 


r  • 

*  .>» 

! 

Jsf- 

jffr 

v 

T:  \ 

w<x 

Fig.  83. — Showing  the   attitude  of  the 
heart  in  a  ease  of  cerebellar  disease. 


DISEASES  OF  THE  BRAIN  265 

patient  by  asking  him  to  stand  on  either  leg  alternately.  By 
this  means  the  greater  weakness  and  unsteadiness  of  the 
affected  side  becomes  apparent. 

Reflexes.  The  deep  reflexes  are  variable :  sometimes 
diminished,  at  other  times  exaggerated,  and  often  changing 
from  day  to  day.  The  superficial  reflexes  are  not  affected, 
and  the  plantar  reflexes  are  always  flexor  in  uncomplicated 
cases.     Sphincter  control  is  not  impaired. 

Cranium.  In  children,  bulging  in  the  occipital  region  over 
the  seat  of  the  tumour  has  been  observed. 

Mental  symptoms  are  absent  until  the  intracranial 
pressure  has  risen  sufficiently  to  cause  impairment  of 
the  cerebral  functions. 

Tumours  of  the  middle  lobe  or  vermis  cerebelli 

The  general  symptoms  are  similar  to  those  already  de- 
scribed under  Tumour  of  the  Lateral  Lobe.  The  ocular 
symptoms  are  bilateral,  and  consist  of  weakness  of  both 
external  recti  muscles,  and  of  the  conjugate  lateral  movements 
of  the  eyes  ;  slow  nystagmoid  jerkings,  equal  in  degree,  occur 
on  looking  to  either  side.  Inco-ordination  and  ataxia  are 
bilateral,  and  most  marked  in  movements  in  the  antero- 
posterior axis.  The  patient  when  standing  may  fall  either 
forwards  or  backwards,  and  also  when  he  suddenly  halts. 
In  some  cases  the  head  is  retracted.  The  gait  is  reeling 
equally  to  either  side,  with  a  sprawling  action  of  the  limbs. 

Owing  to  the  tendency  of  middle-lobe  tumours  to  invade 
the  fourth  ventricle,  internal  hydrocephalus  is  developed 
early  and  indefinite  spastic  symptoms  and  irregular  tremors 
complicate  the  clinical  picture. 

TUMOURS  OF  THE  FOURTH  VENTRICLE 

General  symptoms.  Headache,  vomiting,  and  giddiness, 
develop  early,  and  are  more  or  less  constant.  Headache  is 
usually  occipital  in  position,  and  may  be  associated  with 
stiffness  of  the  neck  and  pain  on  movement.  Optic  neuritis 
may  appear  early  or  late,  but  its  development  is  rapid. 
Giddiness  is  general  in  character  and  un associated  with 
auditory  phenomena.  These  symptoms  may  be  present  for 
one  or  two  years,  and  local  signs  are  strikingly  in  abeyance. 


L>66  NERVOUS  DISEASES 

Loss  of  vision  may  occur  early,  before  the  advent  of  optic 
neuritis,  and  is  probably  due  to  dilatation  of  the  third  ventricle 
causing  direct  pressure  upon  the  optic  chiasnia. 

Mental  changes  are  rarely  in  evidence  until  the  terminal 
stages. 

Examination  of  the  cranial  nerves  reveals  nystagmus  on 
lateral  deviation  of  the  eyes  to  either  side,  similar  in  character 
and  degree ;  paresis  of  both  external  recti  muscles,  weakness 
of  the  conjugate  lateral  movements  of  both  eyes,  and  some- 
times slight  bilateral  weakness  of  the  lower  facial  muscles. 

The  absence  of  interference  with  the  other  cranial  nerves 
is  significant,  although  a  temporary  and  variable  inequality  of 
the  pupils  may  be  observed.  Paralysis  of  the  third  nerve 
with  loss  of  the  pupillary  light  reaction  only  occurs  with 
grave  secondary  complications. 

Motor  system.  Slight  muscular  hypertonicity  associated 
with  inconstant  instability  of  gait  is  a  characteristic  feature 
and  may  persist  for  months.  The  onset  of  definite  spasticity 
and  of  persistent  inco-ordination  indicate  respectively  im- 
plication of  the  pons  and  cerebellum.  As  a  rule  there  is 
no  demonstrable  paresis  of  the  limbs. 

Sensory  system.     There  is  no  alteration  of  sensibility. 

Beflexes.  The  deep  reflexes  are  brisk  or  exaggerated 
equally  on  the  two  sides.  This  may  not  be  accompanied 
by  any  change  in  the  superficial  abdominal  or  plantar 
reflexes. 

The  absence  of  definite  spasticity,  of  paresis,  or  of  changes 
in  the  reflexes,  in  cases  which  at  autopsy  show  not  only 
internal  hydrocephalus,  but  flattening  and  distortion  of  the 
pons  and  medulla,  is  accounted  for  by  the  fact  that  the 
increase  in  pressure  has  been  sufficiently  gradual  to  permit 
the  circulation  in  these  parts  to  adapt  itself  to  the  increased 
intracranial  tension. 

In  some  cases  sugar  is  found  in  the  urine. 

In  the  terminal  stages  death  ensues  from  the  formation  of 
a  pressure  cone  within  the  foramen  magnum,  resulting  in 
respiratory  paralysis. 

Cysticercus  of  the  fourth  ventricle  is  by  no  means  rare, 
and  has  a  slightly  different  symptomatology  from  that  given 
above.  The  distinguishing  features  are :  the  long  duration 
of  the  symptoms  without  definite  physical  signs,  the  sudden 


DISEASES  OF  THE  BRAIN  267 

onset  of  attacks  of  headache,  vomiting  and  giddiness,  the 
association  of  such  attacks  with  sudden  movements  of 
the  head,  the  long  intermissions  with  complete  freedom 
from  all  symptoms,  the  more  frequent  occurrence  of 
glycosuria,  the  late  onset  of  optic  neuritis,  and  the  frequency 
of  sudden  death. 

In  all  cases  presenting  such  symptoms  the  possible 
presence  of  a  tape-worm  {taenia  solium)  should  be  inquired  into 
and  the  risk  of  infection  from  the  mode  of  living  considered. 

The  duration  of  the  symptoms  in  tumour  of  the  ventricle 
varies  from  five  months  to  two  years  ;  but  cases  of  cysticercus 
have  been  known  to  live  for  several  years. 

EXTRA-CEREBELLAR  TUMOURS  (TUMOURS  OF  THE 
CEREBELLOPONTINE  ANGLE) 

Under  this  term  are  included  those  tumours  which  grow 
in  the  posterior  fossa  of  the  skull  between  the  pons  and  the 
cerebellum,  and  originate  commonly  in  connexion  with  the 
eighth  cranial  nerve,  and  less  frequently  with  the  fifth  and 
seventh  nerves.  These  tumours  are  usually  of  firm  consistence 
and  slow  growth,  and  involve  the  pons  and  cerebellum 
secondarily  by  pressure.     (Figs.  84  and  85.) 

In  contradistinction  to  intra-cerebellar  growths,  the  general 
symptoms  are  late  in  appearance.  As  a  rule,  headache  sets 
in  first,  and  is  nearly  always  associated  with  pain  in  the 
neck.  Optic  neuritis  may  be  late  in  appearing  or  absent ; 
when  present  it  is  either  acute  or  subacute  in  degree. 

The  symptoms  produced  by  such  tumours  are  referred 
mainly  to  the  cranial  nerves  implicated  by  the  growth,  and 
in  lesser  degree  to  the  lateral  lobe  of  the  cerebellum  and 
the  pons.  Unilateral  cerebellar  symptoms  are  therefore 
observed  on  the  side  of  the  lesion  (paresis,  ataxia,  and  atonia) 
combined  with  a  spastic  paresis  of  the  opposite  limbs 
from  pressure  upon  the  pyramidal  fibres. 

The  cranial  nerve  symptoms  are  : — 

(a)  Paresis  or  paralysis  of  the  sixth  nerve,  more  complete 
than  in  intra-cerebellar  growths,  and  nystagmus  having  the 
characters  already  described  (p.  263). 

(b)  Paresis  or  paralysis  of  the  fifth  nerve — more  especially 
its  sensory  division. 


268 


NERVOUS  DISEASES 


(c)  The  facial  nerve  is  almost  invariably  affected  at  some 
stage  in .  the  course  of  the  disease,  when  facial  palsy  of  a 
peripheral  type  develops.  It  is  surprising  what  great  and 
long-continued  pressure  may  be  exerted  on  this  nerve  before 
clinical  symptoms  are  produced. 

{d)  Nerve  deafness  on  the  side  of  the  lesion  is  the  earliest, 


Fig.  84. — A  case  of  extra-cerebellar  tumour,  growing  in  the  right 
cerebellopontine  angle,  displacing  and  compressing  the  pons 
and  cerebellum. 

most  definite,  and  most  constant  symptom  of  the  extra- 
cerebellar  growths.  It  may  precede  the  onset  of  the  other 
symptoms  by  months  or  years.  Noises  in  the  ear — tinnitus 
aurium — are  invariably  associated  with  it.  Vertigo  differs 
from  that  of  intra-cerebellar  tumour,  in  that  the  subjective 
feeling  of  rotation  is  always  from  the  sound  to  the  affected 
side. 

(e)  In  rare  instances  the  bulbar  nerves  have  been  impli- 
cated on  the  side  of  the  lesion. 


DISEASES  OF  THE  BRAIN 


269 


Motor  system.  On  the  homolateral  side  a  slight  degree  of 
paresis  associated  with  definite  atonia  and  ataxia  is  present. 
These  symptoms  are  due  to  the  pressure  of  the  tumour  upon 
one  lateral  lobe  of  the  cerebellum.  On  the  contralateral  side 
considerable  paresis  of  a  spastic  type  may  be  present,  owing  to 
pressure  on  the  pyramidal  fibres  above  their  decussation.     In 


Fig.  85. — Bilateral  extra-cerebellar  tumours. 

old  standing  cases,  when  the  pressure  of  the  tumour  is  exerted 
on  both  sides  of  the  pons,  bilateral  spasticity  may  be  observed. 
The  motor  functions  being  affected  in  these  two  ways,  the 
clinical  picture  is  one  of  instability  with  paresis  resembling 
disseminated   sclerosis.      The    gait  is   of  the   ataxic  spastic 


270  NERVOUS  DISEASES 

paraplegic  type:  the  ataxia  being  greater  in  the  homolateral 
leg,  the  spasticity  and  paresis  in  the  contralateral.  (See 
Table,  p.  428.) 

Sensory  system.  Sensory  changes  are  not  present  on  the 
body  or  limbs. 

Reflexes.  The  deep  reflexes  are  increased,  especially  upon 
the  contralateral  side,  where  clonus  may  be  obtained. 

The  superficial  reflexes  are  diminished  on  the  contralateral 
side,  and  associated  with  an  extensor  plantar  response. 
Similar  changes  in  the  superficial  reflexes  of  the  homolateral 
side  are  only  observed  in  the  late  stages. 

The  sphincter  control  may  also  be  interfered  with  in  cases 
with  bilateral  spastic  symptoms. 

TUMOURS  OF  THE  PONS  VAROLII 

The  most  common  tumours  of  this  region  are  glioma  and 
tubercle  ;  sarcoma  and  gumma  may  also  be  found.  Pontine 
tumours  are  more  common  in  children  than  in  adults. 

The  symptoms  may  best  be  described  with  reference  to  the 
two  common  kinds  of  tumour  observed  in  this  locality. 

Glioma.  The  pathological  appearances  presented  by  these 
tumours  led  to  the  use  of  the  term  '  hypertrophy  of  the  pons.' 
The  general  shape  of  the  pons  may  be  preserved,  or  the 
tumour  may  extend  from  the  surface  and  completely  surround 
the  basilar  artery  and  the  emerging  nerves.  The  symptoms 
presented  during  life  are  not  proportionate  to  the  extent 
of  the  pathological  changes  :  a  phenomenon  which  may  be 
explained  by  the  fact  that,  by  Bielschowsky's  method  of 
staining  nerve  fibres,  the  axis-cylinders  of  the  nerve  fibres 
are  not  found  destroyed  by  the  new  growth. 

The  general  symptoms  are  conspicuously  absent.  Head- 
ache only  sets  in  with  the  development  of  internal  hydro- 
cephalus, and  optic  neuritis  is  often  absent  or  only  appears  in 
the  terminal  stages.  Optic  atrophy,  without  obvious  evidence 
of  previous  inflammatory  changes,  has  been  observed  in  quite 
a  number  of  cases.  In  explanation  of  this  it  may  be  suggested 
that  the  slow  increase  of  intra- ventricular  tension,  which  results 
from  the  gradual  compression  and  closure  of  the  aqueduct  of 
Sylvius,  causes  distension  of  the  thin-walled  third  ventricle 
and  pressure  upon  the  optic  tracts.      Cases  in  which   optic 


DISEASES  OF  THE  BRAIN  271 

atrophy  is  observed  are  usually  of  long  standing,  and  in  those 
in  whom  the  symptoms  began  in  early  childhood. 

Vertigo  of  an  indefinite  character  is  almost  always  present 
at  some  stage  of  the  disease. 

Cranial  nerves.  The  first,  third,  and  fourth  nerves  are 
not  affected.  The  fifth  to  the  twelfth  are  all  liable  to  be 
paralysed,  completely  or  incompletely.  The  paralysis  is  often 
bilateral,  and  may  be  of  a  supra-  or  infra-nuclear  type  ; 
when  nuclear,  the  paralyses  are  grouped  according  to  their 
anatomical  relations  within  the  tegmentum  pontis.  For 
this  reason  bilateral  paralysis  of  conjugate  movement  is  a 
common  symptom,  owing  to  the  juxtaposition  of  the  sixth 
nerve  nuclei.  There  may  also  be  found  paralysis  of  different 
nerves  on  opposite  sides— such  as  the  seventh  on  one  side, 
and  the  twelfth  on  the  opposite  side.  The  pupils  may  show 
ectopia,  or  an  eccentric  relation  to  the  iris. 

Motor  system.  Bilateral  paresis,  often  more  marked  on 
one  side,  and  associated  with  spasticity  and  general  ataxia, 
is  usually  present.  Attacks  of  shuddering,  as  if  from  cold, 
are  frequent  and  more  or  less  characteristic  of  tumours  of 
this  region. 

Sensory  system.  Hemianesthesia  is  sometimes  observed, 
but  defects  of  sensibility  may  be  absent  during  the  whole 
course  of  the  disease. 

Reflexes.  The  deep  reflexes  are  increased,  often  unequally ; 
the  superficial  are  diminished  or  absent,  and  the  plantars 
are  extensor  in  type. 

Sphincter  weakness  may  be  delayed  until  a  late  stage. 

The  terminal  stages.  In  children  progressive  hydro- 
cephalus with  loss  of  vision,  defective  hearing,  and  spastic 
paraplegia  ensue ;  cranial  nerve  palsies  are  relatively  slight, 
and  the  mental  state  shows  little  impairment  until  near  the 
end.  Attacks  of  respiratory  failure,  characterised  by  slow, 
periodic,  and  cyclic  respiration,  become  increasingly  frequent, 
and  cause  death  in  the  absence  of  an  intercurrent  complication. 

In  cases  commencing  after  puberty,  although  the  general 
course  of  the  malady  is  similar  to  that  just  described,  the 
clinical  picture  resembles  that  of  a  somewhat  rapid  bulbar 
paralysis  with  difficulty  in  swallowing.  Artificial  feeding 
is  usually  necessary ;  these  cases  die  of  malnutrition  and 
respiratory  failure. 


272  NERVOUS  DISEASES 

Tubercle.  The  pons  may  be  the  seat  of  a  tuberculous 
growth  which  may  be  solitary,  or  one  of  several  scattered 
throughout  the  brain.  The  symptoms  at  the  onset  tend 
to  be  more  acute  than  in  the  gliomatous  cases.  Headache, 
vomiting,  occasionally  optic  neuritis  and  febrile  disturbances 
— associated  with  local  signs  more  or  less  limited — are  also 
points  of  distinction.  Such  general  symptoms  may  subside, 
leaving  merely  the  evidence  of  a  local  lesion.  Such  may  be 
seen  in  paralysis  of  the  seventh  nerve,  or  of  paralysis  of 
lateral  conjugative  movement  on  one  side  with  a  crossed 
hemiplegia,  with  or  without  sensory  loss  ;  or  the  sensory 
division  of  the  fifth  nerve  may  be  paralysed  on  one  side 
with  a  crossed  hemianesthesia,  with  or  without  palsy  of 
the  seventh  nerve. 

This  quiescent  period  may  last  for  a  few  weeks  to  as  many 
months,  only  to  terminate  in  a  gradual  or  sudden  onset  of 
symptoms  of  tuberculous  meningitis. 

TUMOURS  OF  THE  BASE  OF  THE  SKULL 

These  are  relatively  rare,  and  are  usually  sarcomatous  in 
character.  They  are  most  frequently  found  in  the  middle 
and  posterior  fossae,  and  are  of  slow  growth. 

The  symptoms  presented  by  these  cases  are  referred 
chiefly  to  the  cranial  nerves ;  and  local  symptoms  almost 
invariably  precede  by  longer  or  shorter  periods  the  onset  of 
general  symptoms. 

The  symptoms  are  best  described  with  reference  to  the 
position  of  the  new  growth  in  the  three  fossae. 

Tumours  of  the  anterior  fossa.  These  are  rare,  and 
may  be  either  sarcomatous  or  carcinomatous.  They  may 
extend  through  from  the  orbit,  in  which  case  they  are 
recognised  by  the  local  orbital  symptoms  and  unilateral 
anosmia  and  optic  atrophy. 

Tumours  of  the  middle  fossa.  These  are  chiefly  charac- 
terised by  symptoms  referred  to  the  distribution  of  the  fifth 
nerve.  At  the  commencement  complaint  may  be  made  of 
pain  over  one  or  all  of  its  branches,  and  in  the  absence  of 
objective  sensory  loss,  the  condition  may  be  ascribed  to 
trigeminal  neuralgia.  Sooner  or  later,  sensation  becomes 
impaired  over  the  face,  the  corneal  reflex  is  diminished  or 


DISEASES  OF  THE  BRAIN  273 

lost,  and  trophic  ulcers  form  upon  the  cornea.  The  motor 
affection  of  the  fifth  nerve  may  not  develop  until  later.  Not 
infrequently  the  extension  of  the  growth  causes  paralysis  of 
the  third  and  sixth  nerves ;  and  by  pressure  upon  the  optic 
tract  produces  a  homonymous  hemianopsia  to  the  opposite 
side.  The  general  symptoms  "may  remain  in.  abeyance, 
although  in  some  cases  optic  neuritis  and  vomiting  become 
established. 

It  is  important  in  all  such  cases  to  examine  the  hard 
palate,  as  the  growth  may  extend  into  the  mouth  as  well  as 
into  the  cranial  cavity.  Not  infrequently  extension  into 
the  posterior  fossa  may  also  occur,  or  metastatic  deposits 
develop  on  the  opposite  side,  or  indeed  anywhere  on  the 
floor  of  the  skull. 

Subjective  sensations  of  smell  and  taste  arise  from 
secondary  involvement  of  the  uncinate  gyrus,  and  a  slight 
degree  of  hemiplegia  may  be  observed. 

Tumours  of  the  posterior  fossa.  The  symptoms  of 
growths  in  this  locality  are  referred  to  the  sixth,  seventh, 
and  eighth  nerves.  As  the  eighth  is  the  nerve  which  offers 
the  least  resistance,  it  is  first  affected — noises  in  the  ear, 
giddiness,  and  deafness  appearing  early.  Extension  of  the 
tumour  towards  the  foramen  magnum  induces  paralysis  of 
the  ninth,  tenth,  eleventh,  and  twelfth  nerves ;  but  a  forward 
extension  is  not  uncommon,  so  that  the  fifth  nerve  shares 
in  the  paralysis. 

The  symptoms  often  tend  to  become  bilateral  owing  to 
metastatic  deposits  on  the  opposite  side. 

In  addition  to  the  local  symptoms  of  cranial  nerve 
paralysis,  a  slow  and  progressive  spastic  weakness  of  the 
opposite  side  results  from  pressure  upon  the  cms,  pons,  or 
bulb,  and  cerebellar  symptoms  on  the  same  side  may  also 
develop  indirectly  from  pressure. 

The  differential  diagnosis  of  this  condition  from  primary 
tumour  of  the  eighth  nerve  (p.  267)  is  made  by  the  more 
rapid  and  more  extensive  involvement  of  the  other  cranial 
nerves,  by  the  occurrence  and  greater  persistence  of  pain  in 
the  head  and  in  the  distribution  of  the  fifth  cranial  nerve, 
by  the  less  complete  deafness,  by  the  later  onset  of  cerebellar 
symptoms,  and  by  the  paralysis  of  the  external  rectus  muscle 
preceding  the  development  of  nystagmus. 

is 


274  NERVOUS  DISEASES 

The  presence  of  a  tumour  in  the  roof  of  the  mouth,  or 
a  history  of  pre-existent  malignant  disease,  confirms  the 
diagnosis.  Occipital  pain,  especially  on  movement  of  the 
head,  is  a  symptom  of  some  significance  in  these  cases. 

CJeXKKAI.     DlACXOSIS     OF     InTKACKAXIAL     TuiWOUKS 

The  diagnosis  of  an  intracranial  tumour  is  based  upon  the 
study  of  the  symptoms  and  signs  presented  l\y  each  case  — 
phenomena  which  necessarily  vary  according  to  the  nature 
and  situation  of  the  growth — as  detailed  in  the  account  of  the 
regional  diagnosis  just  described. 

Certain  general  symptoms,  however,  are  common  to  intra- 
cranial tumours  and  other  disorders.  These  are  more  par- 
ticularly headache,  vomiting,  optic  neuritis,  and  convulsive 
seizures. 

Persistent  headache,  especially  if  associated  with  vomiting 
and  optic  neuritis,  points  strongly  in  favour  of  a  tumour 
within  the  skull,  but  may  also  be  present  in  meningitis, 
abscess  of  the  brain,  anaBmia,  and  chronic  renal  disease. 

The  existence  of  these  symptoms  without  any  signs  of 
Nephritis  or  of  Ancemia  is  presumptive  evidence  of  an  intra- 
cranial new  growth,  which  will  receive  confirmation  by  the 
presence  of  other  signs — motor,  sensory,  and  reflex — of  organic 
disease  of  the  brain.  On  the  other  hand,  the  absence  of  such 
organic  signs,  but  the  presence  of  obvious  grave  anemia,  or  of 
albuminuria,  cardiac  hypertrophy,  and  arterio-sclerosis,  are 
usually  sufficient  to  explain  the  symptoms.  Cases,  however, 
are  from  time  to  time  seen  in  which  signs  of  organic 
cerebral  disease  are  found  in  association  with  chronic  renal 
disease  or  anaemia.  These  present  great  difficulty  in  diagnosis  ; 
but  the  history  and  the  mode  of  onset  of  the  symptoms, 
or  the  character  and  persistence  of  the  physical  signs,  or 
the  effect  of  treatment,  will  usually  permit  a  decision  being 
made. 

Cases  commencing  with  generalised  convulsive  seizures,  or 
with  attacks  of  minor  epilepsy,  are  difficult  to  distinguish 
from  Idiopathic  Epilepsy.  A  definite  local  aura  as  the  mode 
of  onset  of  the  fit,  and  the  presence  of  a  transient  post-con- 
vulsive hemi-  or  monoplegia,  are  signs  favouring  an  organic 
cause  of  the  seizures  ;  but  it  is  not  until  the  development  of 


DISEASES  OF  THE  BRAIN  275 

a  progressive  paralysis,  or  of  optic  neuritis,  that  the  diagnosis 
of  tumour  can  be  placed  upon  a  firm  basis. 

Symptoms  of  a  similar  nature  may  usher  in  General 
Paralysis  of  the  Insane ;  but  in  this  disease  the  pupillary 
changes,  characteristic  tremor,  and  mental  condition  usually 
indicate  the  true  nature  of  the  malady. 

The  symptoms  of  tumours  of  the  cerebellum  sometimes 
present  a  striking  resemblance  to  those  of  Disseminated 
Sclerosis;  but  in  the  latter  condition,  their  relapsing  character, 
the  presence  of  marked  subjective  paresthesia,  early  sphincter 
trouble,  the  alteration  in  the  reflexes  characteristic  of  a 
pyramidal  affection,  and  the  absence  of  the  general  symp- 
toms of  cerebral  tumour,  usually  suffice  to  distinguish  them. 
(See  Table,  p.  428.) 

The  early  symptoms  of  brain  tumour,  when  occurring  in 
young  women,  may  be  ascribed  to  Hysteria.  It  is  therefore 
most  important  to  look  for  the  existence  of  '  stigmata  '  of 
this  disease— such  as  hemianesthesia,  and  contraction  of  the 
visual  fields  without  organic  changes  in  the  fundus  oculi. 
Optic  neuritis  is  never  present,  nystagmus  does  not  occur, 
and  the  reflexes  do  not  show  the  alterations  found  in  organic 
disease.     (See  Table,  p.  428.) 

Prognosis 

A  few  facts  may  be  given  upon  the  prognosis  of  life  in 
intracranial  tumours.  It  is  obvious  that  in  the  majority 
of  cases  little  more  than  palliation  can  be  rendered  by 
surgical  means.  Some  forms  of  tumour,  either  from  the 
slowness  of  their  growth  or  from  their  situation,  are  more 
amenable  to  surgical  treatment ;  in  these  the  outlook  is 
more  favourable. 

1.  Cerebral  tumours.  In  a  general  way  the  outlook,  as 
regards  the  life  of  a  patient  suffering  from  cerebral  tumour, 
is  not  so  unfavourable  as  in  cerebellar  tumour.  Gummata, 
endotheliomata,  and  fibro-sarcomata  may  be  removed  with  a 
fair  prospect  of  relief  and  prolongation  of  life.  Rapidly 
growing  gliomata  and  sarcomata  are  most  unsatisfactory, 
although  in  favourable  cases  the  duration  of  life  after  operation 
may  be  from  six  months  to  two  years.  In  less  malignant 
forms  life  may  be  prolonged  for  six,  eight,  or  more  years  after 
operation   without  recurrence   of  the   growth.     Tuberculous 

is* 


276  NERVOUS  DISEASES 

tumours  are  unfavourable.  The  prognosis  as  regards  life  is 
bad  in  gliomata,  sarcomata,  and  carcinomata,  if  no  operation 
is  performed. 

The  prognosis  of  meningeal  and  cortical  new  growths  is 
decidedly  more  favourable  than  in  those  which  are  subcortical 
or  deeply  placed. 

2.  Cerebellar  tumours.  In  intra-cerebellar  growths,  not 
submitted  to  operation,  the  prognosis  is  bad ;  but  as  cystic 
degeneration  of  the  growth  sometimes  occurs,  life  may  be 
indefinitely  prolonged,  provided  always  that  the  patient 
survives  the  stage  of  acute  symptoms.  Operations  for  cere- 
bellar tumours,  although  attended  with  considerable  risk,  are 
not  only  justifiable,  but  imperative  as  a  palliative  measure, 
and  on  the  whole  the  results  are  good. 

Extra-cerebellar  tumours  are  usually  of  slow  growth,  and 
even  without  operation  life  may  be  prolonged  for  a  number  of 
years.  The  palliative  operation  may  indefinitely  prolong  life, 
but  the  radical  operation  usually  results  in  death. 

3.  In  pontine  tumours  the  prognosis  depends  upon  the 
site  and  the  rate  of  the  growth.  If  the  bulb  is  involved  or 
compressed,  death  occurs  early.  Intra-ventricular  growths 
are  unfavourable. 

The  possibility  of  sudden  death  in  some  cases  of  intra- 
cranial tumour  should  be  borne  in  mind  in  estimating  the 
prognosis. 

Teeatment 

The  advances  in  cerebral  localisation  have  made  the 
treatment  of  intracranial  tumours  almost  entirely  a  surgical 
matter.  As  soon  as  the  presence  of  an  intracranial  tumour 
has  been  diagnosed,  and  its  position,  if  possible,  localised,  no 
delay  should  occur  in  explaining  the  nature  of  the  malady 
to  the  patient  and  laying  before  him  the  proposed  method  of 
treatment — its  dangers  and  its  advantages. 

A  preliminary  course  of  medicinal  treatment  is  un- 
necessary, except  in  cases  in  which  the  tumour  is  probably 
of  gummatous  nature,  when  a  month  or  six  weeks'  course  of 
energetic  anti- syphilitic  treatment  should  be  carried  out.  If 
at  the  end  of  this  period  the  symptoms  persist,  or  are 
increasing,  no  further  postponement  of  surgical  interference 
is  advisable. 


DISEASES  OF  THE  BRAIN  277 

Surgical  measures  are  undertaken  with  a  twofold  object : 
one,  'palliative,  for  the  relief  of  the  general  symptoms — more 
especially  headache,  vomiting,  and  optic  neuritis ;  the  other, 
radical,  with  a  view  to  the  removal  of  the  growth. 

The  palliative  operations  are  undertaken  with  the  object 
of  relieving  the  excessive  intracranial  tension  present  in  most 
cases  of  tumour.  It  is  not  necessary  that  the  exact  position 
of  the  tumour  should  be  known  before  trephining :  all  that 
is  required  being  a  general  indication,  from  the  extent  and 
degree  of  the  symptoms,  as  to  whether  the  growth  is  above 
or  below  the  tentorium  cerebelli. 

If  the  tumour  is  supra-tentorial  in  position,  the  trephine 
opening  should  be  made  either  in  front  of,  or  behind,  the 
Rolandic  area.  If  the  growth  is  believed  to  lie  behind 
the  motor  area  and  to  be  deep  in  the  subcortical  tissues,  the 
opening  in  the  skull  should  be  made  over  the  occipital  region, 
preferably  on  the  side  of  the  tumour.  If  there  is  no  clear 
evidence  that  the  tumour  is  behind  the  motor  area,  the 
trephine  opening  should  be  made  over  the  frontal  lobe  and 
as  near  the  middle  line  as  possible. 

In  all  cases  a  large  portion  of  bone  should  be  removed  and 
the  dura  mater  freely  incised  by  the  flap  method,  so  as  to 
insure  relief  of  pressure,  freedom  from  strangulation  of  a 
portion  of  brain  through  the  opening  in  the  dura  or  skull, 
and  avoidance  of  subsequent  paralysis. 

When  the  skull  has  been  opened  for  the  relief  of  pressure, 
the  tendency  is  for  the  compressed  brain  around  the  tumour 
to  become  the  seat  of  a  transient  oedema,  and  to  protrude  at 
the  artificial  opening  in  the  skull.  It  is  for  this  reason  that 
the  operation  should  be  carried  out  in  front  of,  or  behind, 
the  motor  area,  according  as  the  tumour  is  supposed  to 
lie  anterior  or  posterior  to  the  Rolandic  fissure.  In  this 
way  the  transmission  of  the  pressure  across  the  motor 
zone  with  consequent  hemiplegia  and  sometimes  aphasia  is 
avoided. 

If  the  growth  is  subtentorial  and  not  localisable  to  one 
or  other  side,  a  large  opening  should  be  made  on  either 
side  of  the  middle  line  over  the  cerebellum ;  but  the  theca 
should  not  be  opened  in  the  first  stage,  as  the  removal  of  the 
bone  alone  affords  sufficient  temporary  relief,  and  enables  the 
parts  to  gradually  accommodate  themselves  to  the   altered 


278  NERVOUS  DISEASES 

pressure  conditions.  At  a  later  stage  the  dura  mater  may  be 
opened  on  both  sides,  or  only  on  one  side,  if  the  tension  Bhows 
great  unilateral  excess.  By  this  method  the  serious  risk  of 
collapse  from  sudden  relief  of  pressure  is  obviated.  Moreover, 
it  affords  an  opportunity  of  radical  interference  at  the  second 
stage. 

The  effect  of  such  palliative  operations  is  sometimes 
remarkable.  Headache  and  vomiting  are  relieved,  optic 
neuritis  and  epileptic  seizures  gradually  subside,  and  stupor 
or  coma  gives  place  to  a  brighter  and  clearer  mental  con- 
dition. 

The  outstanding  complication  of  palliative  trephining  lies 
in  the  tendency  towards  hernia  cerebri. 

Palliative  operation  only  is  recommended  in  cases  of 
sarcoma  of  the  base  of  the  skull,  and  in  tumours  of  the 
auditory  nerve.  Although  the  latter  are  in  many  respects 
most  suitable  for  extirpation,  the  risks  of  their  removal  have 
in  the  majority  of  cases  proved  far  too  serious,  and  the 
permanent  condition  of  the  patient  is  not  materially 
improved. 

Cerebellar  tumours  are  frequently  cystic,  and  tapping, 
without  removal,  may  be  resorted  to  with  success. 

Badical  operations,  with  a  view  to  the  removal  of  the 
new  growth,  ought  to  be  undertaken  only  when  the  tumour 
is  clearly  and  definitely  localised.  For  this  purpose  the 
trephine  opening  is  made  over  that  part  of  the  brain  to 
which  the  localising  symptoms  point  as  the  position  of  the 
tumour. 

The  importance  of  a  large  opening  is  great,  as  the  surgeon 
is  enabled  to  see  and  define  the  limits  of  the  tumour  and  the 
point  of  maximum  pressure. 

If  the  tumour  grows  from  the  dura  mater,  it  may  be 
removed  at  once  along  with  the  membrane  to  which  it  is 
attached.  If  it  is  within  the  brain,  the  parts  should  be 
carefully  inspected  and  its  limits  defined.  If  it  is  obvious 
that  the  growth  is  extensive  and  of  an  infiltrating  nature, 
no  attempt  should  be  made  to  remove  it.  If  no  tumour 
is  visible,  the  brain  may  be  carefully  incised  and  the 
subcortical  tissue  examined.  If  a  growth  of  small  size  is 
found,  it  may  be  removed  provided  that  its  removal  will 
not  increase  paralysis  or  lead  to  aphasia. 


DISEASES  OF  THE  BRAIN  279 

Chapter   VI 

JNTRACK ANIA L   ABSC ESS 

Suppuration  within  the  cranial  cavity  may  implicate  both 
membranes  and  the  brain.  When  it  affects  the  membranes 
it  may  be  either  generalised,  as  in  suppurative  meningitis,  or 
localised,  as  in  extra-  and  intradural  abscess.  Suppuration 
may  also  affect  the  brain  and  membranes  suppurative 
meningo-encephalitis. 

Etiology.  Intracranial  suppuration  is  the  result  of 
local  and  general  infection  by  staphylococci,  streptococci,  and 
pneumococci. 

The  local  infections  are  due  to  the  following  causes :  — 

1.  Traumata  of  the  scalp,  skull,  membranes,  and  brain, 
in  consequence  of  compound  fractures,  gunshot,  or  other 
injuries.  Intracranial  abscess  may  also  arise  as  the  result 
of  contrecoup—Qi  blow  on  one  side  of  the  skull,  causing 
injury  to  the  brain  on  the  opposite  side— which  may 
become  the  seat  of  abscess.  An  injury  to  the  scalp  alone 
may  give  rise  to  abscess.  Such  injuries  may  be  followed 
by  suppuration  within  thirty-six  hours ;  or  after  four  or 
five  weeks'  interval.  On  the  other  hand,  all  symptoms 
may  be  deferred  for  a  number  of  years — up  to  twenty 
years,  in  one  of  our  cases.  An  abscess  may  be  situated 
superficially  or  deeply.  When  superficial,  symptoms  arise 
early;  when  deep  the  occurrence  of  symptoms  may  be 
delayed. 

2.  Suppurative  otitis  media.  Intracranial  abscess  may 
be  secondary  to  both  acute  and  chronic  suppuration  in 
the  middle  ear.  The  routes  by  which  infection  may  be 
carried  are : — 

(a)  Through  the  roof  of  the  middle  ear  and  antrum. 
When  carried  by  this  route  suppuration  is  found  in  the 
middle  fossa  of  the  skull,  involving  the  temporo-sphenoidal 
lobe  and  its  meningeal  coverings. 

(b)  Through  the  posterior  wall  of  the  antrum,  the  labyrinth 
and  the  internal  auditory  meatus.  Lateral  sinus  thrombosis 
and  cerebellar  abscess  are  commonly  brought  about  by 
infection  along  this  route. 


280  NERVOUS  DISEASES 

(c)  Through  the  venous  and  lymphatic  channels  between 
the  ear  and  the  cranial  cavity,  and  by  the  eighth  nerve. 

The  most  frequent  intracranial  complications  of  acute  otitis 
media  are  suppurative  meningitis  and  sinus  thrombosis ; 
those  of  chronic  otitis  are  temporo-sphenoidal  and  cerebellar 
abscess,  but  both  meningitis  and  sinus  thrombosis  may  also 
occur. 

3.  Disease  of  the  nose  and  accessory  cavities.  The  intra- 
cranial complications  due  to  these  causes  may  result  from 
operative  interference  upon  the  nose  or  from  acute  and 
chronic  suppuration  of  the  sinuses.  Infection  may  occur 
directly  from  carious  bone  or  indirectly  through  the  orbit, 
optic  nerve,  and  ophthalmic  veins,  or  by  the  venous 
and  lymphatic  channels.  Disease  of  the  ethmoidal  and 
sphenoidal  sinuses  is  most  prone  to  give  rise  to  intracranial 
suppuration. 

It  may  be  stated  in  general  terms  that  acute  infective 
disorders  of  the  ear,  nose,  and  accessory  sinuses,  operative 
procedures  and  injury  are  liable  to  give  rise  to  acute 
suppurative  meningitis,  whereas  chronic  infections  are 
more  prone  to  induce  abscess  of  the  brain. 

4.  Other  local  infective  causes  of  cerebral  abscess  are — 
tuberculous  and  syphilitic  affections  of  the  cranial  bones, 
acute  osteomyelitis  of  the  skull,  carbuncle,  "and  erysipelas  of 
the  scalp. 

5.  The  general  infections  which  give  rise  to  intracranial 
suppuration  are  :  pyaBmia,  bronchiectasis,  empyaima,  gangrene 
of  the  lung,  peritonitis  and  pericarditis,  abscess  of  the  liver, 
appendicitis,  suppuration  of  joints  and  of  the  tonsils,  acute 
fevers — such  as  cerebro-spinal  meningitis,  enteric,  scarlet 
fever,  measles,  and  influenza — streptothrix,  and  actino- 
mycosis. 

A  so-called  idiopathic  abscess  of  the  brain  has  been 
described.  The  causes  of  this  are  frequently  to  be  found  in 
an  old  and  forgotten  scalp  wound,  a  healed  ear  suppuration, 
a  nasal  focus  of  disease,  previous  cerebro-spinal  meningitis, 
erysipelas,  and  influenza.  The  majority  of  idiopathic  abscesses 
are  in  reality  due  to  old  suppurative  ear  disease  which  may 
have  escaped  detection. 

As  the  causes  which  give  rise  to  intracranial  suppuration 
are  numerous,  the  initial  symptoms  are  varied  and  complex, 


DISEASES  OF  THE  BRAIN  281 

being  in  part  those  of  the  causal  condition,  and  in  part  those 
of  the  intracranial  complication. 

Pathology.  Brain  abscess  may  be  either  acute  or  chronic. 
In  the  acute  form  pus  is  found  occupying  a  cavity  which  may 
or  may  not  have  any  definite  separation  from  the  surrounding 
brain  substance,  which  is  soft  and  pultaceous.  The  abscess 
is  usually  in  the  white  matter,  and  is  separated  from  the 
surface  of  the  brain  by  what  appears  to  be  healthy  tissue. 
In  this  type  of  abscess  infection  has  taken  place  through 
the  vascular  and  lymphatic  channels. 

In  the  more  common,  or  chronic  abscess,  adhesions  have 
formed  between  the  affected  bone,  the  dura,  the  pia- 
arachnoid  membranes  and  the  cerebral  cortex.  Owing  to 
the  lesser  resistance  offered  by  the  white  matter,  pus  makes 
its  way  more  readily  in  the  subcortical  tissues ;  so  that 
abscesses  of  this  character  are  of  mushroom  shape,  the  stalk 
being  attached  to  the  dura  mater  at  the  seat  of  infection. 
They  are  usually  encapsulated  by  a  wall  of  dense  tissue. 
They  may  rupture  either  into  the  subarachnoid  space  or 
into  the  ventricles. 

Heiman l  records  the  relative  frequency  of  abscess  in 
different  localities  as  follows :  Out  of  818  cases  of 
intracranial  suppuration,  539  were  cerebral  and  279 
were  cerebellar  abscess.  In  the  cerebral  cases  the 
temporal  lobe  was  much  the  most  commonly  affected, 
being  involved  seven  times  more  often  than  the  rest  of 
the   cerebrum. 

When  due  to  direct  extension  from  ear  disease,  abscess  of 
the  temporal  lobe  is  situated  in  the  third  temporo-sphenoidal 
gyrus,  or  between  the  base  of  the  lobe  and  the  convexity. 
The  abscess  rarely  occurs  in  the  anterior  part  of  the  lobe. 
It  may  be  superficial  with  a  local  meningitis,  or  healthy 
brain  may  be  present  over  the  abscess,  or  the  abscess  may 
be  deeply  placed  in  the  lobe. 

The  majority  of  cerebellar  abscesses  are  found  near  the 
groove  containing  the  sigmoid  sinus.  They  are  laterally 
placed  when  infection  is  through  the  sinus,  and  mesially 
when  infection  is  through  the  labyrinth. 

Pontine  and  crural  abscesses  are  rare. 

The  two  sides  of  the  brain  are  equally  affected. 

1  Heiman,  Zeitchr.f.  OkrenlwilJc,  vol.  xxxii. 


282  NERYOlTS  DISEASES 

Traumatic  and  otitic  abscesses  are  usually  single,  and 
metastatic  abscesses  are  multiple. 

Symptoms.  The  symptoms  of  intracranial  abscess  are 
both  general  and  local :  the  latter  varying  according  to  the 
part  of  the  brain  directly  implicated,  or  to  pressure  upon 
nerves  or  structures  situated  at  some  distance  from  the  seat 
of  the  disease. 

General  symptoms.  The  general  symptoms  are,  in  the 
main,  similar  to  those  produced  by  tumour,  or  other  cause  of 
increased  intracranial  pressure.  Headache  is  usually  an  early 
and  sometimes  a  prominent  symptom.  It  is,  however,  by  no 
means  a  constant  feature,  nor  does  it  invariably  correspond 
to  the  site  of  the  abscess.  There  is  no  necessary  relation 
between  the  seat  of  the  abscess  and  localised  tenderness  on 
cranial  percussion. 

Mental  symptoms  are  frequently  early  as  well  as  constant 
features  in  most  cases.  The  most  marked  is  a  tendency  to 
somnolence  or  lethargy,  with  excessive  lassitude,  loss  of  the 
power  of  attention,  and  inability  to  answer  questions  or  to 
carry  on  conversation.  Concurrently  with  the  progress  of 
the  disease,  the  lethargy  deepens,  and  the  stupor  becomes 
more  pronounced  until  it  terminates  in  unconsciousness  and, 
finally,  coma. 

The  temperature  varies  from  a  normal,  or  slightly  sub- 
normal, curve  to  one  showing  moderate  pyrexia.  A  sudden 
rise  to  104°  or  105°  F.  would  indicate  rupture  of  the  abscess 
into  the  ventricle.  In  cases  complicated  with  sinus  thrombosis, 
the  chart  is  of  the  pysemic  type  with  temperature  rising  daily 
to  105  or  106°  F.  and  dropping  to  a  normal  or  subnormal 
level  with  rigors  and  profuse  perspiration. 

The  pulse  is  usually  slow  and  regular,  and  even  when  the 
temperature  is  raised,  a  corresponding  increase  in  the  pulse 
frequency  is  not  observed. 

Optic  neuritis  is  sometimes  present,  but  when  it  occurs 
it  is  a  late  symptom.  Many  cases  of  cerebral  abscess 
pass  through  their  whole  course  without  the  appearance  of 
this  sign. 

Vomiting  is  an  early,  but  not  a  constant  symptom.  In 
cerebellar  abscess  its  persistence  is  more  marked  than  in 
abscess  in  other  localities. 

Eapid  and  pronounced  emaciation  and    cachexia   are  not 


DISEASES  OF  THE  BRAIN  283 

uncommon  symptoms  of  cerebral  abscess,  and  wben  present 
are  very  characteristic. 

In  all  cases  of  chronic  purulent  otorrhoca,  the  onset  of 
headache,  with  irritability  or  any  other  sign  suggesting  an 
alteration  in  the  patient's  normal  mental  condition,  an 
attack  of  causeless  vomiting,  or  a  slight  degree  of  pyrexia, 
should  arouse  suspicions  as  to  the  likelihood  of  intra- 
cranial suppuration.  A  sudden  acute  increase  in  the 
discharge  from  the  ear,  or  a  sudden  arrest  of  a  chronic 
otorrhoea  ought,  if  accompanied  by  symptoms  such  as 
have  been  described,  to  be  looked  upon  as  signals  of  a 
dangerous  kind. 

Although  three  stages  are  usually  described  in  the  develop- 
ment of  the  symptoms  of  intracranial  abscess,  it  is  impossible 
to  define  their  limits,  as  one  is  merely  an  intensification  of 
that  which  precedes  it,  and  in  turn  merges  into  that  which 
follows.  The  first  stage,  however,  may  be  described  as  that 
of  the  prodromal  phenomena— irritability,  change  of  tem- 
perament sometimes  attributed  to  hysteria,  slight  pyrexia, 
headache,  and  one  or  more  attacks  of  causeless  vomiting ; 
the  second  stage  as  that  of  the  full  development  of  the 
abscess,  with  a  slow  pulse — sometimes  pyrexia,  at  other  times 
a  subnormal  temperature — headache,  and  a  tendency  to 
lethargy  and  somnolence.  This  merges  into  the  terminal 
stage  of  stupor  and  coma. 

Cerebrospinal  fluid.  This  may  present  a  milky  appearance 
when  withdrawn  by  lumbar  puncture.  A  cytological  examina- 
tion may  reveal  the  presence  of  polymorpho-nuclear  cells  with 
or  without  the  presence  of  micro-organisms.  The  latter  may 
also  be  present  without  any  leucocytes. 

Localising  symptoms.  The  temporo-sphenoidal  lobe  and 
the  lateral  lobe  of  the  cerebellum  are  the  two  commonest 
situations  of  cerebral  abscess. 

An  alteration  in  the  percussion  note  of  the  skull  has  been 
stated  to  be  a  sign  of  cerebral  abscess.  Over  the  abscess  the 
percussion  note  becomes  clear  and  high-pitched  when  the 
head  is  struck  with  a  percussion  hammer.  This  is  best 
detected  by  aid  of  a  stethoscope  placed  upon  the  forehead, 
or  on  a  bald  patch. 

Temporo-sphenoidal  abscess.  The  general  symptoms  already 
described  are  present,  on  whichever  side   the   abscess  may 


28 1  NERVOUS  DISEASES 

be  situated ;  but  should  the  suppuration  be  present  in 
the  left  lobe  in  right-handed  persons,  a  form  of  partial 
aphasia  has  been  found  as  one  of  its  earliest  signs.  This 
consists  of  an  inability  to  name  objects,  although  a  know- 
ledge of  their  function  is  still  retained  and  may  be 
expressed. 

An  early  sign  of  especial  significance  is  loss  or  impairment 
of  the  epigastric  and  abdominal  reflexes  on  the  side  opposite 
the  abscess ;  this  may  be  found  even  without  any  sign  of 
motor  or  sensory  paralysis. 

Cerebellar  abscess.  Symptoms  pointing  to  involvement  of 
the  lateral  lobe  of  the  cerebellum  are :  retraction  of  the 
head  and  rigidity  of  the  neck,  giddiness  often  so  pronounced 
that  the  head  is  with  difficulty  raised  from  the  pillow,  and 
a  reeling  or  staggering  gait.  Some  patients  assume  in  bed 
a  characteristic  attitude,  lying  on  the  side  with  the  legs  flexed, 
and  that  aspect  of  the  face  upwards,  which  corresponds 
to  the  side  of  the  abscess.  Slow  and  large  range  nystagmus 
to  the  side  of  the  abscess,  and  inco-ordination  of  the 
movements  of  the  limbs  upon  the  same  side,  are  the  chief 
localising  signs. 

The  localising  symptoms  of  abscess  of  other  parts  of  the 
brain  do  not  differ  from  those  described  under  Intracranial 
Tumours. 

Prognosis.  Acute  abscess  is  a  serious  malady,  which 
unless  treated  surgically  will  cause  the  death  of  the  patient 
within  a  few  weeks.  If  the  abscess  is  uncomplicated  and 
operation  for  its  evacuation  undertaken  early,  the  prognosis 
is,  on  the  whole,  favourable.  This  differs  to  some  extent 
upon  the  seat  of  the  abscess  :  temporo-sphenoidal  abscess 
presenting  a  more  favourable  outlook  than  that  situated  in 
the  cerebellum. 

The  prognosis  in  chronic  abscess  varies.  If  of  small  size, 
its  presence  may  be  undetected,  providing  it  does  not  give 
rise  to  localising  symptoms.  When  it  gives  rise  to  general 
symptoms,  the  prognosis  is  grave,  and  the  condition  calls  for 
immediate  surgical  interference. 

Diagnosis.  The  differential  diagnosis  of  abscess  of  the 
brain  requires  to  be  made  from  suppurative  meningitis  and 
infective  sinus  thrombosis.  The  points  in  the  diagnosis  are 
given  in  the  Table  upon  p.  288. 


DISEASES  OF  THE  BRAIN  285 

Treatment.  The  prophylatic  treatment  consists,  in  trau- 
matic cases,  in  rendering  aseptic  all  damaged  tissues. 
Where  the  bone  is  injured  and  dirty,  the  injured  portion 
should  be  removed.  If  the  dura  mater  or  brain  be  injured, 
the  former  should  be  freely  exposed  and  opened  to  permit  the 
inspection  of  the  lepto- meninges  and  brain ;  and,  where 
necessary,  the  infected  portions  should  be  removed.  When 
the  brain  has  been  injured  it  is  advisable  not  to  close  the 
wound,  but  to  drain  for  a  time. 

Any  local  suppurative  disease  of  the  accessory  cranial 
sinuses  should  be  attended  to,  and  operation  for  its  removal 
effected  without  delay. 

All  chronic  otorrhceas  require  careful  attention  and  treat- 
ment. Macewen1  gives  the  following  indications  for  opening 
the  mastoid  antrum: — (a)  Repeated  inflammations  in  the 
mastoid  antrum  and  cells  with  swelling  over  the  mastoid 
process  or  a  fistulous  opening  in  the  bone.  (6)  An  acute 
inflammation  of  the  mastoid  antrum  and  cells  with  retention 
of  pus.  (c)  When  there  are  initial  symptoms  of  intra- 
cranial involvement  associated  with  chronic  purulent  otorrhoea. 

(d)  Persistent  chronic  otorrhoea,  arising  from  carious  disease 
of  the  temporal  bone,  which  has  resisted  ordinary  treatment. 

(e)  If  the  discharge  is  highly  offensive  or  contains  virulent 
pathogenic  organisms. 

EXTEADUEAL    AND    LsTTEADUEAL    ABSCESSES 

An  abscess  may  form  between  the  infected  bone  and  the 
dura  mater.  This  is  rare,  except  in  cases  of  ear  disease. 
This  form  of  abscess  may  not  give  rise  to  any  specific  signs— 
being  found  by  the  surgeon  during  the  course  of  an  operation 
for  mastoid  disease.  It  is  not  infrequently  in  direct  com- 
munication with  the  tympanic  cavity,  and  may  give  signs  of 
its  presence  by  the  welling  out  of  pus  from  the  bone  after 
this  cavity  has  been  cleansed. 

An  intradural  abscess  may  also  be  present,  the  pus  being 
prevented  from  infecting  the  subarachnoid  space  by  the 
formation  of  adhesions  during  the  slow  process  of  meningeal 
infection.  The  symptoms  of  this  type  of  abscess  are  similar 
to  those  of  abscess  within  the  brain. 

1  Macewen,  Pyogenic  Diseases,  1S93. 


286  NERVOUS  DISEASES 


IM'KCTIVE   SINUS   THROMBOSIS. 

This  is  an  infective  or  inflammatory  condition  occurring 
mainly  in  adult  persons.  It  is  of  local  origin,  and  affects  the 
sinus  nearest  the  site  of  the  primary  disease.  In  contrast  to 
the  marasmic  form  it  is  found  mainly  in  the  bilateral  sinuses. 
In  the  later  stages  it  is  associated  with  meningitis  and 
pysemic  abscesses  of  the  brain. 

Etiology.  The  common  cause  is  a  chronic  suppurative 
inflammation  of  the  middle  ear,  with  a  cario-necrotic  affection 
of  the  surrounding  bone,  infecting  the  sigmoid  sinus.  It 
may  also  arise  from  trauma  of  the  skull,  and  from  infective 
diseases  in  the  orbit,  nose,  and  tonsils ;  from  erysipelas  of 
the  face  and  scalp,  dental  caries  with  periostitis  and 
retropharyngeal  abscess. 

It  may  be  due  either  to  direct  extension,  or  to  a  thrombus 
in  and  extending  from  a  small  neighbouring  vein.  The 
result  of  this  infection  is  the  gradual  formation  of  a 
thrombus  within  usually  the  sigmoid  vein.  This  in  time 
becomes  infected,  and  gives  rise  to  a  general  infection 
of  the  system  through  suppuration  in  and  disorganisation 
of  the  clot. 

The  local  infection  may  extend  downwards  into  the 
jugular  vein,  or  upwards  into  the  cavernous  sinus.  A 
general  systemic  infection  may  also  occur  directly  from 
the  ear  without  the  intervention  of  a  localised  sinus 
phlebitis. 

Symptoms.  The  symptoms  are  both  general  and  local : 
the  former  being  those  of  pyaemia,  and  the  latter  depending 
upon  the  particular  sinus  affected. 

The  general  symptoms  are  vomiting  and  headache — the 
latter  usually  severe,  and  either  widespread  or  referred  to 
the  seat  of  the  disease. 

The  temperature  is  remittent,  rising  and  falling  rapidly, 
associated  with  a  small  rapid  and  irregular  pulse,  profuse 
perspirations  and  rigors.  The  tongue  is  dry  and  coated ; 
anorexia  is  common,  and  diarrhoea  more  frequent  than 
constipation.  Optic  neuritis  may  also  be  present.  Macewen 
has  described  two  types  of  pysemic  infection :  (1)  the 
pulmonary — whose  symptoms  are  cough,  pain  in  the  chest, 


DISEASES  OF  THE  BRAIN  287 

dark-coloured  sputum,  and  fetor  of  breath ;  and  (2)  the 
abdominal — characterised  by  a  dry  cracked  tongue,  vomiting, 
tympanites,  diarrhoea,  or  the  typhoid  state. 

The  local  symptoms  point  to  thrombosis  of  particular 
sinuses. 

(a)  The  superior  longitudinal  sinus.  Thrombosis  of  this 
sinus  is  usually  a  marasmic  condition  characterised  by  oedema 
of  the  scalp  and  distension  of  the  veins,  epistaxis,  convulsions, 
and  more  rarely  paralysis. 

(b)  The  cavernous  sinus.  Both  sinuses  are  usually  in- 
volved as  a  result  of  infective  disorders  of  the  orbit,  face, 
mouth,  nose,  throat,  and  pharynx.  The  symptoms  consist  of 
pain  over  the  supra-  and  infra-orbital  branches  of  the  fifth 
nerve,  protrusion  of  the  eyeballs,  oedema  of  the  eyelids  and 
side  of  the  nose,  ptosis  and  ocular  palsies.  Optic  neuritis 
may  also  be  present. 

(c)  Tlie  sigmoid  sinus.  The  symptoms  may  be  of  only  slight 
intensity.  When  present  they  consist  of  oedema  and  pain 
over  the  mastoid  bone,  phlebitis  and  thrombosis  in  the 
internal  jugular  vein  and  deep  veins  of  the  neck,  pain  on 
pressure  over  the  upper  part  of  the  jugular  vein,  which  may 
be  palpable,  and  sometimes  enlargement  of  and  suppuration 
in  the  cervical  glands. 

The  differential  diagnosis  has  to  be  made  mainly  from 
abscess  of  the  brain  and  suppurative  meningitis.  The  leading 
points  of  difference  are  given  in  the  Table  on  p.  288. 

Prognosis.  This  is  essentially  unfavourable.  If  surgical 
interference  is  carried  oat  in  the  early  stages,  the  outlook  is 
more  hopeful.  The  duration  of  an  untreated  case  is  only 
a  few  weeks. 

Treatment.  Treatment  is  entirely  surgical,  and  limited 
to  the  affections  of  the  sigmoid  sinus.  It  consists  in  exposing, 
clearing  out,  and  ligaturing  the  affected  sinus  or  vein,  as  well 
as  removing  the  primary  source  of  infection.  Owing  to  the 
occasional  coexistence  of  abscess,  attention  should  be  given 
to  the  cerebellum  and  the  temporo-sphenoidal  lobe. 


288 


NERVOUS  DISEASES 


DIFFERENTIA!    Diacnosis  of  Srrrri;  vn\  k   M  knimhtis.  V\  i;ii;i;  \i.  Ansrioss, 
am'  [nfective  Sims  Thrombosis, 


Menisi;iiis. 

Abscess. 

Infective  Thrombosis. 

( Inset 

Rapid 

Relatively  slower 

Rapid 

Temperature 

Pyrexia,  100°  Falir.  to 
104°  Fahr. 

Normal,    subnormal, 

or  slight  pyrexia 

Pysemic     type     with 

sudden  rises  and 
falls  accompanied 
by  rigors,  ^ve. 

Pulse 

Rapid,  irregular,  and  in- 
termittent 

Slow  and  regular 

Rapid  and  irregular 

Focal  .signs 

Twitchings    of   limbs, 
strabismus,    rigidity 
of     neck,      Kemig's 
sign  present 

Signs     of     lesion    of 
temporo-spbenoidal 
lobes,    or    of    cere- 
bellum 

Mastoid  tenderness 
and  swelling  j  ten- 
derness along  jugu- 
lar vein ;  exophthal- 
mos on  one  side ; 
cedema  of  scalp 

Blood 

Leucocytes  from  17,000 
to  21,000  per  c.mm. 

Leucocytes   may  not 
exceed  14,000 

Leucocytes  about 

20,000 

Cerebrospinal 

fluid 

Purulent  or  semi-puru- 
lent under  increased 
pressure,  large  poly- 
mo  rplio-  nuclear 
counts.  Strepto-,  sta- 
phylo  -  and  pneuino- 
cocci  present 

May  be  milky.     Poly- 
mo  rpbo-nu  clear 
cells  may  be  present. 
Micro-organisms 
may      be     present 
without  leucocytes 

Same  as  abscess 

Chapter  VII 


HYDROCEPHALUS 

This  is  a  condition  characterised  by  distension  of  the 
ventricles  of  the  brain  with  cerebro-spinal  fluid.  It  arises 
in  three  ways  :  — 

1.  As  a  congenital  or  developmental  condition,  either  alone, 
or  in  association  with  defects  — such  as  spina  bifida,  hydro- 
niyelia,  or  other  malformations. 

2.  As  a  primary  condition,  coming  on  at  any  period 
after  birth. 

3.  As  a  secondary  condition,  resulting  from  obstruction 
and  damming  up  of  the  cerebro-spinal  fluid  within  the 
ventricles. 

Etiology.  The  causes  of  primary  hydrocephalus  are  not 
clearly  understood. 


DISEASES  OF  THE  BRAIN  283 

It  would  appear  to  be  due  to  an  inflammatory  affection  of 
the  lining  membrane  of  the  ventricles,  more  especially  that 
covering  the  choroid  plexus.  In  two  cases — the  one  a  child, 
the  other  an  adult — the  ventricular  ependyma  was  studded 
with  small,  hard,  whitish  nodules  composed  of  proliferated 
ependymal  cells,  with  evidence  of  old  inflammatory  lesions 
in  the  choroid  plexus.  The  presence  of  thickening  of 
the  intima  of  the  choroidal  vessels  suggested  a  syphilitic 
origin. 

The  secondary  forms  arise  in  connexion  with  tumours  of 
the  brain,  especially  when  subtentorial  and  affecting  the 
ventricular  systems.  Subsequent  inflammatory  changes 
cause  blocking  of  the  veins  of  Galen,  with  resulting 
hyperemia  of  the  choroid  plexuses.  Most  frequently  it  arises 
from  meningitis  obstructing  the  communication  between 
the  ventricular  system  and  the  spinal  subarachnoid  spaces, 
especially  in  posterior  basic  and  epidemic  cerebro- spinal 
meningitis.     It  is  rare  in  tuberculous  meningitis. 

Symptoms.  (1)  Congenital  hydrocephalus.  The  most 
striking  feature  is  enlargement  of  the  head.  This  may  be 
present  at  birth,  or  be  observed  to  take  place  a  few  weeks 
after  birth.  The  enlargement  is  limited  to  the  cranial  vault, 
the  bones  of  which  become  separated  sometimes  to  a  sur- 
prising extent.  Owing  to  this  the  forehead  is  abnormally 
prominent,  and  the  parietal  region  directed  backwards.  In 
comparison  to  the  large  head  the  face  appears  small  or 
shrunken,  and,  owing  to  the  displacement  of  the  orbital 
plates  of  the  frontal  bones,  the  eyes  are  turned  downwards. 
The  veins  of  the  scalp  may  be  enormously  distended.  The 
sutures  of  the  skull  are  widely  open. 

In  association  with  the  cranial  enlargement  a  number  of 
nervous  symptoms  may  be  present.  These  consist  of  con- 
vulsions, attributable  to  the  abnormal  pressure  exerted  upon 
the  cerebral  cortex  against  the  calvarium  by  the  enlarged  and 
distended  ventricles ;  paralysis  and  spasticity  of  the  limbs, 
with  muscular  contractures,  exaggeration  of  the  deep  reflexes, 
and  extensor  plantar  responses  are  common.  The  mental 
symptoms  are  those  of  marked  mental  enfeeblement  or 
amentia. 

Vision  is  frequently  impaired  from  pressure  of  a  distended 
third  ventricle  upon  the  optic  chiasma,  or  from  optic  atrophy. 

19 


290  NERVOUS  DISEASES 

Nystagmus  and  convergent  strabismus  are  accompanying 
symptoms. 

As  this  condition  is  usually  congenital  or  arises  in  early 
life,  its  recognition  is  easy. 

2.  Primary  hydrocephalus.  When  it  occurs  in  adults  it  is 
difficult  to  recognise.  It  is  liable  to  be  confused  with  cerebral 
tumour  or  intracranial  aneurism.  It  would  appear  to  be 
one  of  the  conditions  giving  rise  to  'pseudo-tumour.'  The 
symptoms  are  those  of  intracranial  tumour  in  so  far  as 
headache,  vomiting,  optic  neuritis,  and  giddiness  are  concerned. 
These  are  often  attended  by  convulsions,  either  general  or 
focal.  The  important  symptoms  in  favour  of  primary  internal 
hydrocephalus  are  the  early  impairment  of  upward  movement 
of  the  eyes,  and  of  the  light  reflex,  at  a  stage  when  the  optic 
neuritis  is  acute  and  the  vision  unimpaired,  especially  as 
regards  the  perception  of  light.  Early  failure  of  vision  often 
occurs  when  the  ophthalmoscopic  appearances  are  characteristic 
of  acute  papillitis  and  without  visible  evidence  of  atrophy. 

In  the  convulsions  with  local  onset  the  commencement  is 
not  always  in  the  same  locality :  one  seizure  beginning  in  the 
arm  and  another  in  the  leg.  This  variability  in  the  local 
seizures  is  important  in  distinguishing  the  condition  from  a 
localised  tumour. 

Besides  the  convulsive  phenomena  the  motor  system 
exhibits  a  progressive  spasticity,  which  is  bilateral  and  equal 
on  the  two  sides,  and  associated  with  slight  general  weak- 
ness. The  reflexes  are  characteristic  of  upper  motor  neurone 
lesion. 

3.  Secondary  hydrocephalus.  The  symptoms  and  signs  of 
the  secondary  or  obstructive  form  are  grafted  upon  those  of 
the  pre-existing  cerebral  lesion. 

In  cases  of  acute  meningitis  enlargement  of  the  head 
may  be  noticed;  but  in  the  early  stages,  at  all  events,  it  is 
quite  impossible  to  differentiate  the  symptoms  of  the  two 
conditions. 

Where  hydrocephalus  is  secondary  to  slowly  growing  intra- 
pontine  tumours  or  chronic  meningitis,  in  addition  to  enlarge- 
ment of  the  head,  which  depends  upon  the  amount  of 
ossification  of  the  cranial  bones,  a  change  in  the  percussion 
note  of  the  skull  may  be  observed,  often  associated  with  the 
development  of  an  intracranial  murmur. 


DISEASES  OF  THE  BRAIN  291 

The  nervous  phenomena  are  headache,  convulsions  usually 
generalised  but  sometimes  local,  spasticity  of  varying  degrees 
associated  with  increase  of  the  deep  reflexes,  diminution  of 
the  superficial  abdominal  reflexes,  and  the  presence  of  the 
extensor  type  of  plantar  response. 

Very  characteristic  are  the  ocular  phenomena.  These  are 
(1)  early  loss  of  upward  movements  of  the  globes,  associated 
with  (2)  diminution  or  loss  of  the  pupillary  light  reflex — 
presumably  due  to  the  pressure  of  a  distended  third  ventricle 
upon  the  third-nerve  nucleus;  and  (3)  failure  of  vision  without 
or  with  only  slight  optic  neuritis,  going  on  to  optic  atrophy 
from  pressure  upon  the  chiasma. 

In  rapidly  growing  intracranial  tumour  with  acute 
pressure  symptoms,  hydrocephalus  is  less  extreme  and  optic 
neuritis  more  pronounced,  and  the  symptoms  generally  are 
those  of  increased  intracranial  pressure. 

The  diagnosis  is  not  difficult  in  infants  when  enlarge- 
ment of  the  head  has  taken  place.  The  enlarged  head  of  the 
rachitic  child  differs  from  that  of  the  hydrocephalic  by  its 
squarer  shape,  by  the  absence  of  prominent  fontanelles,  and  by 
the  other  associated  signs  of  rickets. 

When  hydrocephalus  arises  as  a  primary  condition  in 
later  childhood  or  early  adult  life,  greater  difficulty  may 
be  experienced  in  diagnosis,  as  the  symptoms  are  more 
comparable  to  those  of  intracranial  tumour. 

The  diagnosis  of  this  condition  rests  first  upon  the  presence 
of  severe  general  symptoms  without  associated  localising 
signs  ;  secondly,  upon  the  characteristic  development  of  the 
ocular  and  visual  symptoms  ;  and  thirdly,  upon  the  onset  of 
bilateral  spasticity. 

Lumbar  puncture  is  often  of  value  in  the  diagnosis  of 
primary  hydrocephalus — a  highly  albuminous  fluid  being 
withdrawn. 

Prognosis.  In  the  majority  of  cases  of  developmental 
hydrocephalus  the  outlook  is  bad,  and  death  ensues  within  a 
comparatively  short  time.  A  few  cases  of  mild  and  non- 
progressive character  may  live  for  a  number  of  years  with 
impaired  mental  capacity. 

In  the  acquired  cases  the  prognosis  is  bad.  In  some 
instances,  on  the  other  hand,  the  malady  is  arrested  and  life 
is  prolonged  with  impaired  mental  faculties  and  epilepsy. 

19  * 


292  NERVOUS  DISEASES 

In  the  t}Tpe  arising  in  consequence  of  cerebral  tumour, 
or  of  the  meningitides,  the  prognosis  is  that  of  the  causal 
condition. 

Treatment  applies  almost  solely  to  the  developmental 
form  with  marked  enlargement  of  the  head.  The  pressure  of 
bandages  or  of  a  plaster  applied  to  the  head  is  an  old  method, 
and  may  be  tentatively  adopted.  Tapping  the  ventricles 
and  lumbar  puncture  are  of  little  use— the  former  being, 
moreover,  a  highly  dangerous  procedure.  Ligature  of  the 
internal  carotid  arteries  has  been  of  use  in  some  cases. 

A  method  of  establishing  a  communication  between  the 
ventricular  and  subarachnoid  spaces  has  been  adopted  with 
some  success  by  Cheyne  and  Sutherland. 

In  the  primary  and  secondary  forms,  trephining  with  a 
view  to  decompression  should  be  performed  on  both  sides  of 
the  skull  over  the  inferior  and  post-parietal  regions.  Repeated 
lumbar  puncture  might  also  be  of  some  service  in  relieving 
intracranial  tension. 


Part  VII 

DISEASES   OF   THE   MEMBRANES 

EPIDEMIC    CEREBRO-SPINAL   MENINGITIS 
(CEREBROSPINAL  FEVER) 

This  is  an  acute  infective  inflammation  of  the  pia-  arach- 
noid membrane  over  the  brain  and  spinal  cord,  due  to  the 
presence  of  the  meningococcus,  or  diplococcus  intracellularis 
meningitidis.  It  occurs  in  both  an  epidemic  and  a  sporadic 
form,  and  there  is  reason  to  suppose  that  the  condition  known 
as  posterior  basic  meningitis  is  a  mild,  or  atypical  example  of 
the  same  disease. 

Etiology.  Numerous  epidemics  of  cerebro- spinal  fever 
have  occurred  during  the  past  century;  but  attention  has 
been  recently  redirected  towards  it  owing  to  severe  epidemic 
outbreaks  in  New  York  in  1905,  in  Glasgow  in  1906,  and  in 
Belfast  in  1907.  Epidemics  of  cerebro- spinal  fever  seem  to 
differ  from  those  of  scarlet  fever,  or  of  smallpox,  by  the 
irregularity  of  their  distribution — the  disease  cropping  up  in 
disconnected  places.  The  majority  of  cases  occur  in  young 
people  under  fifteen  years  of  age — the  first  quinquennium 
showing  the  greatest  number  of  cases.  Adult  life,  however, 
does  not  confer  complete  immunity,  although  it  is  rare  over 
twenty-five  or  thirty  jrears  of  age.  The  epidemics  have 
appeared  chiefly  during  the  winter  and  spring  months,  the 
number  of  cases  sensibly  diminishing  during  the  summer  to 
reappear  in  the  late  autumn  and  winter. 

An  epidemic  may  be  preceded,  or  succeeded,  by  some  in- 
crease in  the  number  of  the  sporadic  cases.  In  the  intervals 
between  epidemics  small  accessions  to  the  average  number  of 
cases  usually  observed  during  the  winter  have  been  noted,  but 
not  amounting  to  an  epidemic.  Posterior  basic  meningitis, 
mainly  met  with  in  infants  under  twelve  months,  and  by  some 

293 


294  NERVOUS  DISEASES 

regarded  as  a  sporadic  form  of  this  disease,  has  also  been 
found  to. occur  in  epidemic  form. 

The  disease  is  favoured  by  over- crowding  and  insanitary 
conditions,  as  it  rarely  shows  itself  outside  the  poorer  quarters 
of  large  cities.  It  is  not  communicable  as  a  rule  from  one 
person  to  another,  as  are  the  ordinary  forms  of  infectious 
disease. 

The  meningococcus.  The  essential  cause  of  the  disease  is 
the  diplococcus  intracellularis  meningitidis.  It  is  difficult  to 
cultivate  and  of  low  vitality.  It  is  found  in  the  polymorpho- 
nuclear cells  of  the  meningeal  exudation.  It  has  also  been 
found  in  the  secretions  of  the  nose,  mouth,  and  conjunctiva, 
which  would  appear  to  be  its  normal  habitation.  It  is 
probable,  but  by  no  means  certain,  that  it  effects  an  entrance 
into  the  subdural  space  through  the  lymphatics  of  the 
tonsils  and  the  sphenoidal  sinuses. 

Morbid  anatomv.  The  meninges  of  the  brain  and  spinal 
cord  present  an  inflamed  appearance  and  are  covered  with 
a  purulent  exudation.  This  is  especially  seen  over  the  base 
with  an  extension  upwards  to  the  convexity.  The  cerebro- 
spinal fluid  is  increased  in  quantity,  and  of  turbid  appearance. 
The  ventricles  may  also  be  distended,  and  their  walls  red  and 
cedematous. 

Microscopically,  the  pia-arachnoid  membrane  is  found 
to  be  the  seat  of  pronounced  inflammatory  changes,  the 
blood-vessels  being  engorged  and  surrounded  by  a  profound 
perivascular  cell  infiltration.  In  chronic  cases,  or  in  cases 
observed  after  the  acute  stage  has  passed  away,  the  pia 
arachnoid  shows  considerable  cicatrical  thickening.  The 
nerves,  both  cranial  and  spinal — but  especially  the  former 
— are  similarly  affected,  and  may  be  involved  in  the 
cicatrical  tissue. 

Symptoms.  Although  a  period  of  incubation  is  probable, 
there  are  no  well-determined  facts  regarding  it.  In  the 
majority  of  cases  the  disease  comes  on  more  or  less  suddenly 
with  headache,  restlessness,  and  vomiting.  The  other  nervous 
symptoms  at  this  stage  vary  with  the  intensity  of  the 
disease,  but  may  consist  of  extreme  restlessness,  irritability 
amounting  to  delirium,  convulsions  and  coma,  ending  rapidly 
in  death  in  a  few  hours  in  severe  instances  (fulminant  type). 

In  the  less  acute  cases  more  obviously  meningeal  symptoms 


DISEASES  OE  THE  MEMBRANES 


295 


are  observed — such  as  twitchings  of  the  face,  arms  and 
legs,  squinting,  and  photophobia.  Amongst  other  symptoms 
detected  in  well-defined  cases  are  retraction  of  the  head  ;  or  if 
less  severe,  rigidity  of  the  muscles  of  the  neck  on  attempts  to 
bend  the  head,  pain  at  the  back  of  the  head  and  in  the  neck, 
or  a  tendency  to  opisthotonos.  Kernig's  sign  may  be  present. 
This  consists  of  an  inability  to  extend  the  leg  upon  the  thigh 


Fig. 


>. — Photograph  of  a  child  presenting  Kernig's  sign.     Xote  the 
contraction  of  the  hamstring  muscles. 


when  the  child  is  lying  on  the  back,  with  the  thigh  in  a 
semi-flexed  position.     (Fig.  86.) 

There  may  be  intervals  of  consciousness,  during  which  com- 
plaint is  made  of  severe  headache — frontal  or  occipital;  and 
pain  along  the  spinal  column  is  a  frequent  symptom.  In  favour- 
able cases  the  symptoms  gradually  subside,  with  the  exception 
of  the  head  retraction,  which  may  persist  for  a  long  time. 

The  knee  joints  are  sometimes  swollen  and  painful,  and 
there  is  a  state  of  general  hyperesthesia,  so  that  the  child 
cannot  be  moved  or  even  touched  without  inducing  great 
pain  and  discomfort. 

There  is  no  temperature  curve  characteristic  of  the  disease. 
According  to  Osier  it  may  assume  forms :  (a)  differing  little 
from  that  of  an  average  case  of  enteric  fever  ;  (b)  resembling 


296  NERVOUS  DISEASES 

tuberculosis  in  its  remissions  and  exacerbations ;  and  (c) 
suggestive  of  malaria. 

The  jmlse  is  rapid  and  irregular,  in  which  it  differs  from 
that  of  tuberculous  meningitis,  which  is  habitually  slow. 

Paralysis  of  the  cranial  nerves  may  be  severe,  and  is 
sometimes  permanent  even  in  the  cases  which  recover. 
Most  common  is  paralysis  of  the  oculo-motor  nerves  with 
diplopia.  Loss  of  vision  is  sometimes  found.  Deafness 
also  occurs  partly  from  a  complicating  otitis  media  as  well 
as  from  damage  to  the  auditory  nerve.  A  slight  degree  of 
facial  paralysis  has  also  been  found,  but  more  as  an  early 
and  transient  than  as  a  permanent  symptom. 

Mental.  Mild  dementia  has  been  observed  to  persist 
after  the  acute  symptoms  of  delirium  and  stupor  have  passed 
away.  This  has  been  attributed  to  an  accumulation  of  fluid 
within  the  ventricles,  and  may  be  relieved  by  lumbar  puncture. 
The  symptom  tends  to  become  permanent  with  chronic  hydro- 
cephalus, mental  impairment,  and  sometimes  epileptic  fits. 

There  is  also  a  chronic  type  of  the  malady,  lasting  for 
many  weeks,  with  remissions  and  exacerbations  of  fever, 
and  corresponding  improvement  or  relapse  of  the  nervous 
symptoms,  delirium,  stupor,  and  coma.  In  these  cases  the 
patient  may  be  reduced  to  a  state  of  profound  marasmus 
and   extreme  emaciation. 

The  rash.  In  its  true  form  this  would  appear  to  be  a  rare 
condition.  It  consists  of  hemorrhagic  spots,  appearing  in 
crops,  over  various  parts  of  the  body — such  as  the  thighs,  legs, 
hands,  abdomen,  and  chest.  They  may  also  be  seen  in  the 
eyelids  and  under  the  conjunctivae.  They  appear  early  in  the 
disease.  The  spots  vary  in  size  from  a  pin-point  to  that  of 
a  sixpenny-piece,  are  of  a  deep  purple  colour,  and  do  not 
disappear  upon  pressure.  Other  forms  of  this  skin  eruption 
are  seen  in  a  roseola  like  that  of  enteric  fever,  and  herpes 
may  occur  in  various  parts  of  the  body. 

The  blood.  There  is  a  well-marked  polyrnorpho-nuclear 
leucocytosis  in  all  acute  cases,  sometimes  amounting  to  50,000 
per  c.mm.  In  the  chronic  cases  this  is  not  observed.  It  is  of 
little  value  as  a  means  of  differentiating  this  malady  from 
tuberculous  meningitis. 

The  cerebro-spi?ial  fluid.  This  is  usually  under  consider- 
able pressure,  and  is  opacme,  turbid,  or  purulent.    A  cytological 


DISEASES  OF  THE  MEMBRANKS  297 

examination  reveals  a  large  number  of  polymorphonuclear 
leucocytes,  which  are  much  in  excess  of  the  lymphocytes. 
In  tuberculous  meningitis  the  mononuclear  cells  preponderate. 
Suitable  staining  will  reveal  the  diplococcus  lying  within  the 
polymorpho-nuclear  cells,  but  occasionally  outside  them.  It 
is  found  as  a  rule  during  the  persistence  of  the  disease. 

Diagnosis.  The  diagnosis  has  to  be  made  from  the 
other  forms  of  meningitis.  The  chief  points  are  given  in  the 
Table  upon  p.  304. 

Prognosis.  In  the  acute  or  fulminant  form  death  occurs 
in  a  few  days.  In  the  less  acute  form  recovery  ensues  in  a 
small  proportion  of  the  cases,  while  in  the  chronic  relapsing 
type,  recovery  may  be  expected  in  a  certain  number  of  cases. 

The    outlook,    however,    is   in    all    cases   of   this   disease 
obviously  grave.    In  the  event  of  recovery,  permanent  paralyses  . 
of  some  of  the  cranial  nerves     especially  the  auditory  and  the 
optic— are  found,  and  the  mental   condition  may  pass  into 
one  of  mild  dementia. 

The  duration  of  the  disease  varies  from  a  few  days  in  the 
severe  cases  to  several  months  in  the  chronic  types. 

Treatment.  Treatment  should  be  conducted  along  general 
lines,  with  abundant  nourishment,  and  stimulants  administered 
when  necessary.  Cold  applications  locally  to  the  head  and 
spine  may  give  comfort,  while  frequent  bathing  at  a  temperature 
of  100°  F.,  and  perhaps  cupping  over  the  neck,  may  relieve 
distress.  Sedatives — such  as  the  bromides  and  chloral — 
may  be  advantageously  used,  and  hypodermic  injections  of 
morphia  have  been  recommended  by  some  writers,  but  would 
seem  harmful  in  many  cases. 

Lumbar  puncture,  with  the  removal  of  any  large  quantity 
of  cerebro-spinal  fluid,  is  not  likely  to  be  of  much  benefit  in 
the  early  and  acute  cases,  unless  there  are  signs  of  a  rapid 
rise  in  the  intraventricular  pressure,  but  may  be  of  temporary 
service  in  the  more  prolonged  and  relapsing  cases. 

The  indication  for  puncture  lies  in  an  increase  of  the 
pressure  in  the  subarachnoid  and  ventricular  spaces.  As  the 
fluid  is  expressed  from  the  needle  in  a  considerable  stream  in 
many  of  these  cases,  marked  benefit  will  be  temporarily 
obtained  by  this  operation.  If,  on  tapping,  no  fluid  comes 
away,  or  the  stream  is  so  small  as  to  suggest  diminution  of 
pressure,  lumbar  puncture  should  not  be  further  attempted. 


298  NERVOUS  DISEASES 


POSTBRIOB  BASIC  MENINGITIS 

This  disease  is  probably  a  sporadic  form  of  cerebro-spinal 
meningitis.  It  may  occur  at  any  season.  It  affects  both 
sexes  equally.  It  is  most  common  in  the  first  year  of  life,  but 
also  occurs  in  later  years.  It  is  due  to  the  presence  of  the 
diplococcus  intracellularis  in  the  subarachnoid  space,  which  is 
probably  infected  through  the  posterior  nares. 

Pathology.  The  morbid  changes  are  more  strictly  con- 
fined to  the  posterior  basal  portions  of  the  brain  than  in 
cerebro-spinal  meningitis.  The  purulent  exudation  is  found 
chiefly  in  the  interpeduncular  space  and  upon  the  anterior 
surface  of  the  pons  and  crura  cerebri.  It  tends  to  spread 
along  the  base  of  the  temporo-sphenoidal  lobes  towards  the 
ventricles,  so  as  to  cause  blocking  of  the  foramen  of  Majendie, 
and  eventually  internal  hydrocephalus. 

Symptoms.  The  onset  is  acute  or  subacute.  The  child 
becomes  peevish  and  irritable.  Convulsions  and  vomiting  may 
ensue,  and  it  is  noticed  that  movements  of  the  head  cause  pain. 
In  a  few  hours  or  days  the  malady  becomes  fully  developed. 
The  child  then  lies  with  its  head  retracted  and  sometimes  in  a 
state  of  extreme  opisthotonos.  Cranial  nerve  paralysis, 
especially  of  the  sixth  nerve,  may  be  present,  and  there 
may  be  difficulty  in  swallowing. 

This  state  of  irritation  gives  place  to  one  of  stupor  and 
coma.  If  death  does  not  ensue,  a  slow  process  of  recovery  sets 
in.  The  mental  condition  improves  and  the  rigidity  becomes 
progressively  less.  Kernig's  sign,  which  is  present  early  in 
these  cases,  eventually  disappears. 

Blindness  is  observed  in  some  cases  on  recovery  from  the 
acute  stage.  As  a  general  rule  this  is  a  transient  symptom, 
although  it  may  persist  for  as  long  as  six  months.  On 
examination  the  fundus  oculi  is  normal  and  the  pupillary 
light  reaction  is  unaffected.  In  other  cases,  where  blindness  is 
permanent,  the  pupillary  reaction  is  lost,  and  the  examination 
may  reveal  atrophy  of  the  optic  disc.  When  such  is  not 
present,  obvious  evidence  of  hydrocephalus  is  found. 

The  after-condition  depends  upon  the  degree  of  obstruc- 
tion to  the  flow  of  the  cerebro-spinal  fluid  through  the 
foramen  of  Majendie.  If  complete  obstruction  has  taken 
place,   secondary   hydrocephalus    results,    which  may  either 


DISEASES  OF  THE  MEMBRANES  299 

result  in  death  at  a  late  stage,  or  in  mental  and  physical 
impairment. 

The  cerebrospinal  fluid  is  under  pressure  and  turbid,  and 
contains  an  excess  of  polymorpho-nuclear  cells.  The  diplo- 
coccus  intracellularis  is  present,  especially  in  the  early  stages. 

The  differential  diagnosis  is  found  in  the  Table  upon  p.  304. 

The  treatment  consists  of  repeated  lumbar  puncture  and 
the  withdrawal  of  cerebro-spinal  fluid. 

TUBERCULOUS  MENINGITIS 

This  is  most  common  in  children  during  the  period  of  the 
first  dentition.  It  is  also,  though  less  commonly,  found  in 
adults.  In  quite  young  children,  especially  under  one  year, 
the  non-tuberculous  form  of  meningitis,  or  posterior  basic 
meningitis,  is  more  common. 

It  is  doubtful  whether  tuberculous  meningitis  is  ever  a 
primary  condition,  though  the  source  of  infection  may  be 
difficult  to  find.  In  two  cases  the  only  evidence  of  tubercle,  after 
careful  examination,  was  in  one  instance  a  small  caseous 
bronchial,  and  in  the  other  a  small  caseous  mesenteric  gland. 
The  commonest  source  of  infection  is  tuberculous  disease  of 
glands  ;  then,  in  order  of  frequency,  tuberculous  disease  of  the 
lungs,  joints,  bones,  testicles,  and  the  ear.  A  solitary  tuber- 
culous tumour  within  the  brain  may  be  a  source  of  infection. 
Operations  for  tuberculous  disease  of  the  spine  are  particularly 
liable  to  set  alight  the  meningeal  inflammation. 

Morbid  anatomy.  In  the  earliest  stages  the  membranes 
of  the  base  may  present  a  greasy  or  sticky  appearance ;  but 
in  the  later,  when  meningitis  is  well  developed,  small  white 
nodules,  varying  from  the  size  of  a  pin-point  to  a  pin-head,  are 
seen  studded  over  the  meninges,  along  the  fissure  of  Sylvius, 
the  fissure  between  the  cerebellum  and  cerebrum,  and  along 
the  lines  of  the  great  vessels.  The  exudation  is  of  a  serous, 
sero-fibrinous,  or  fibrino-purulent  character.  The  tubercles 
are  formed  of  collections  of  small  round  cells  in  the  peri- 
vascular sheaths  of  the  smaller  arteries.  In  some  cases  giant 
cells  and  caseation  in  various  stages  are  present. 

The  arteries  may  exhibit  more  or  less  acute  endarteritis. 

The  brain  itself  is  infiltrated  with  small  round  cells, 
especially  along  the  vessels,  and  the  cortical  cells  and  fibres 
show  softening  and  degenerative  changes. 


300  NEKVOUS  DISEASES 

In  most  cases  the  ventricles  are  distended  and  sometimes 
contain  a  turbid  fluid.  The  ependyma  may  be  granular,  and 
the  choroid  plexus  is  distended  and  tortuous,  and  may  be 
covered  by  an  exudation.  The  sub-ependymal  tissues  are 
frequently  softened.  In  many  cases  the  foramen  of  Majendie 
is  blocked,  and  this  may  give  rise  to  ventricular  distension.  In 
other  cases  the  veins  of  Galen  are  obstructed.  Owing  to  intra- 
ventricular distension,  general  flattening  of  the  convolutions 
of  the  convexity  is  observed.    The  dura  mater  is  not  affected. 

Symptoms.  The  mode  of  onset  is  usually  insidious  ;  more 
rarely  it  is  acute.  When  the  latter,  it  is  a  terminal  com- 
plication of  active  tubercle  elsewhere.  The  symptoms  vary 
somewhat  according  to  the  age  of  the  patient.  In  children, 
when  the  onset  is  insidious,  there  is  a  well-defined  prodromal 
stage  lasting  from  two  up  to  twelve  weeks.  This  is  shown  by 
general  malaise,  and  occasionally  slight  headache.  The  child 
is  languid  and  irritable,  and  often  has  a  dull  frowning 
expression.  Constipation  and  a  foul  breath,  accompanied  by 
occasional  vomiting  at  night  without  obvious  cause,  are 
common  accompaniments. 

At  this  period  the  temperature  may  be  slightly  raised  up 
to  100°  F.  or  101°  F.,  and  the  pulse  quickened. 

The  further  course  of  the  disease  may  be  for  descriptive 
purposes  divided  into  the  stages  of  irritation  and  of  paralysis. 

In  the  irritative  stage  the  symptoms  assume  an  acuter 
phase,  which  is  due  to  the  development  of  a  definite 
meningeal  lesion.  These  are  headache  (often  severe),  gene- 
ralised convulsions,  and  rigidity  of  the  neck  without  marked 
retraction  of  the  head.  The  child  may  lie  upon  its  side  with 
the  limbs  drawn  up  and  the  abdomen  retracted.  A  general 
hyper-sensitiveness  to  touch,  to  movement  of  the  limbs,  and 
to  light,  may  be  present.  The  temperature  and  pulse  rate 
fall  at  this  period,  and  the  latter  is  frequently  irregular.  As 
this  stage  progresses,  local  paraly tic  symptoms  begin  to  appear. 
The  pupils,  which  may  have  been  dilated,  become  irregular 
and  variable  in  size ;  paralysis  of  one  or  other  ocular  muscle 
may  result  in  squint  and  diplopia ;  and  irregular  nystagmoid 
movements  of  the  eyeballs  are  observed.  The  convulsions 
tend  to  become  more  localised,  although  they  may  appear  in 
different  parts  of  the  body  at  different  times.  A  condition  of 
general    rigidity — at   first    slight    in    degree — develops,    and 


DISEASES  OF  THE  MEMBRANES  301 

weakness  or  paresis  of  one  limb  or  of  one  side  of  the  body 
and  aphasia  may  become  distinct.  All  these  symptoms  are 
more  or  less  transient,  but  improvement  is  never  continuous. 

In  the  later  phases  of  this  stage,  rigidity  becomes  more 
marked,  convulsions  more  generalised,  and  the  pupils  are 
inactive  to  light.  These  symptoms  are  due  to  the  develop- 
ment of  internal  hydrocephalus,  with  distension  of  the 
ventricles.  The  mental  condition  is  one  of  stupor,  though 
irritability  is  seen  when  the  patient  is  moved.  The  irritative 
merges  into  the  second  or  paralytic  stage.  Here  the  pulse 
is  quickened,  soft,  and  less  irregular.  The  stupor  deepens, 
difficulty  in  swallowing  comes  on,  with  more  complete  and 
persistent  paralysis  of  the  limbs.  Independent  wandering 
movements  of  the  eyeballs  are  also  seen.  The  corneal  reflexes 
are  lost,  the  superficial  reflexes  impaired,  and  in  older  patients 
an  extensor  plantar  response  is  obtained.  Eetention  of  urine 
in  the  early  stage  or  incontinence  later,  Cheyne- Stokes 
respiration,  increasing  coma,  and  an  occasional  convulsion 
terminate  the  scene,  which  is  sometimes  accompanied  by  a 
considerable  rise  of  temperature. 

Optic  neuritis  develops  during  the  irritative  stage,  but  is 
rarely  severe  in  type.  Tubercles  of  the  choroid  have  been 
observed,  and  are  described  by  some  writers  as  one  of  the 
diagnostic  signs  of  the  disease. 

Sugar  may  be  present  in  the  urine  in  the  later  stages. 

In  cases  with  an  acute  onset,  active  tuberculous  disease 
is  found  elsewhere  in  the  body.  Here  sudden  headache, 
vomiting,  convulsions,  and  delirium  or  stupor,  with  rigidity 
of  the  neck  and  cranial  nerve  palsies,  are  found. 

In  adults,  the  headache  is  in  excess  of  somnolence.  The 
clinical  picture  is  more  like  that  of  an  intracranial  tumour, 
although  the  meningeal  symptoms  suffice  usually  to  distinguish 
the  two  conditions.  On  the  other  hand,  it  is  not  infrequent 
for  meningitis  to  be  grafted  upon  the  symptoms  of  an  already 
existent  tuberculous  tumour. 

The  cerebrospinal  fluid  may  be  turbid  when  withdrawn, 
and  on  examination  is  found  to  contain  the  tubercle  bacillus. 
The  mononuclear  cells  or  lymphocytes  preponderate  over  the 
polymorpho-nuclear  cells. 

The  blood  shows  a  leucocytosis,  but  not  to  the  same  extent 
as  in  cerebro-spinal  meningitis.     Calmette's  reaction  is  not  of 


302  NERVOUS  DISEASES 

much  assistance  in  diagnosis,  as  it  may  be  present  in 
association  with  tuberculous  disease  in  any  part  of  the  body. 

Diagnosis.  In  the  early  stages  of  the  disease,  when  the 
symptoms  are  vague  and  indefinite,  it  is  next  to  impossible 
to  state  what  the  course  of  the  malady  may  be.  When  more 
acute  symptoms  make  their  appearance  -  and  especially  in 
adult  cases— the  diagnosis  has  usually  to  be  made  from  enteric 
fever  and  apical  pneumonia.  The  almost  universal  adoption 
of  precise  methods  of  diagnosis  in  doubtful  cases  is  nowhere  of 
more  value  than  in  these  conditions.  A  lymphocytosis  of  the 
cerebro- spinal  fluid,  especially  if  associated  with  the  presence 
of  tubercle  bacilli  in  the  centrifugalised  deposit,  is  conclusive 
evidence  of  tuberculous  meningitis  ;  while  the  Widal  reaction 
of  the  blood  would  establish  the  existence  of  enteric  fever. 
From  lobar  pneumonia  the  diagnosis  is  made  by  the  auscul- 
tatory signs  present  in  that  disease.     (See  Table,  p.  304.) 

Prognosis.  The  disease  is  invariably  fatal  within  two  or 
three  weeks  from  the  onset  of  the  initial  symptoms.  The 
cases  which  have  been  reported  as  cured  have  probably  been 
sporadic  instances  of  cerebro-spinal  meningitis  rather  than 
the  tuberculous  variety. 

Treatment.  This  is  conducted  on  general  principles.  The 
patient  should  be  kept  in  bed  in  a  darkened  room,  the  head 
shaved,  and  an  ice-bag  applied  to  it.  Symptoms  ought  to  be 
treated  as  they  arise— more  especially  the  constipation,  which 
is  a  feature  of  the  malady.  A  lumbar  puncture,  done  at  the 
outset  in  order  to  establish  the  diagnosis  by  an  examination 
of  the  cerebro-spinal  fluid,  may  have  effected  a  transitory 
improvement  in  the  symptoms ;  but  it  is  unlikely  that  a 
repetition  of  this  operation  will  lead  to  any  permanent  benefit. 

SUPPURATIVE  MENINGITIS 

Etiology.  This  is  an  infection  of  the  lepto-meninges  by 
streptococci,  staphylococci  and  pneumococci.  Pneumococcus 
meningitis  may  occur  in  consequence  of  acute  middle  ear 
disease,  and  probably  also  as  a  sporadic  condition.  The 
sources  of  infection  are  the  same  as  those  already  described 
under  Abscess  of  the  Brain,  p.  279.  They  are  :  traumata  of 
the  head,  suppurative  middle  ear  disease,  disease  of  and 
operations  upon  the  nose  and  accessory  canities,  acute  fevers 


DISEASES  OF  THE  MEMBRANES  303 

^such  as  pneumonia,  influenza,  typhoid  fever,  scarlet  fever, 
and  measles — and  sometimes  a  terminal  infection  in  renal, 
cardiac,  and  pulmonary  diseases. 

Meningitis  is  also  described  as  a  result  of  infection  by  the 
gonococcus  and  the  bacillus  coli. 

Symptoms.  The  onset  of  acute  cerebral  symptoms  such 
as  headache,  delirium,  and  convulsions  in  association  with 
pyrexia,  rapid  pulse,  and  vomiting,  occurring  in  the  course  of 
any  of  the  above-mentioned  conditions  is  suggestive  of  a 
secondary  inflammation  of  the  pia-arachnoid  membrane. 

The  headache  is  severe  and  continuous,  and  is  accompanied 
by  delirium  passing  into  stupor.  The  temperature  runs  a 
more  or  less  continuous  course,  not  rising  and  falling  as  in 
pysemic  conditions  or  sinus  thrombosis. 

The  pulse  is  rapid  at  first,  but  eventually  becomes 
intermittent  and  finally  slow. 

Patients  in  this  condition  are  hyperaBsthetic  to  light,  to 
sounds,  and  to  handling  of  the  limbs  and  to  movement. 
Muscular  twitchings  and  retraction  of  the  neck  are  also 
frequent. 

Localising  symptoms  may  be  present,  according  to  the 
position  of  the  initial  inflammation. 

In  meningitis  of  the  base  of  the  brain,  oculo-motor  palsies, 
strabismus,  twitchings  of  the  facial  muscles,  and  sometimes 
optic  neuritis  are  observed.  In  meningitis  of  the  convexity, 
mono-  or  hemiplegic  weakness  may  develop,  and  in  the  later 
stages  convulsions  of  a  generalised  character. 

In  whatever  part  of  the  brain  the  inflammation  commences, 
the  general  symptoms  as  described  above  progress,  and  the 
stupor  deepens  into  coma,  in  which  the  patient  dies. 

The  cerebrospinal  fluid  varies  from  an  opalescent  to  a 
yellowish  or  truly  purulent  fluid.  The  pressure  of  the  fluid 
is  raised,  and  as  much  as  15  or  20  c.cm.  may  be  readily 
withdrawn.  Albumen  is  present  in  considerable  quantity. 
The  polymorpho-nuclear  cells  are  much  increased.  Cultiva- 
tions reveal  the  presence  of  staphylococci,  streptococci,  and 
pneumococci. 

Diagnosis.  The  presence  of  polymorpho-nuclear  cells 
alone  does  not  prove  the  existence  of  suppurative  meningitis  : 
as  they  may  be  present  without  meningitis — in  brain 
abscess,  suppurative  labyrinthitis,  and  sinus  phlebitis.    Micro- 


:;.u 


NERVOUS  DISEASES 


organisms  have  also  been  found  alone  in  the  fluid  in  sinus 
phlebitis,  septicamia,  and  brain  abscess. 

Suppurative  meningitis  should  only  be  definitely  diagnosed 
when  a  large  number  of  polymorpho-nuclear  cells  are  present 
along  with  the  above-mentioned  organisms  in  the  cerebro- 
spinal fluid. 

The  course  of  the  disease  is  rapid,  and  invariably  fatal. 
According  to  the  intensity  of  the  symptoms  it  varies  from  a 
few  days  to  two  or  three  weeks.  Its  ordinary  course  may  be 
complicated  by  the  existence  of  a  sinus  thrombosis  or  of 
abscess  of  the  brain. 

Treatment  is  of  little  avail  once  the  meninges  have  become 
infected.  Eest,  the  application  of  ice  to  the  head,  purgation 
and  sedatives,  may  be  prescribed  with  temporary  relief.  The 
abstraction  of  a  quantity  of  cerebro-spinal  fluid  has  been 
followed  by  temporary  improvement. 


Table  giving  the  Points  of  Differential  Diagnosis  between  the  several 
Forms  of  Meningitis  above  described 


Cerebrospinal 
Fever. 

Postbasic 

JlKXIXGITIS. 

TUBERCULOUS 

Meningitis. 

Suppurative 
Meningitis. 

Age  at 
onset 

Children  and   young 
adults 

Infants  under  one 
year 

Children  &  adults 
under  50  years 

All  ages 

Infection 

Primary,     sporadic, 
epidemic 

Primary  sporadic 
epidemic 

Secondary  to  tu- 
bercle       else- 
where 

Secondary  to  frac- 
tures, operations, 
or      suppurative 
disease  elsewhere 
in  body 

Organism 

Diplococcus       intra- 
cellularis 

Diplococcus    in- 
tracellularis 

Tubercle  bacillus 

Streptococcus, 
staphylococcus, 
pneumococcus 

Cytology 
of     cere- 
bro-spiDal 
fluid 

Polymorpho-nuclears 
largely   in    excess 
of  lymphocytes 

Polymorpho-nu- 
clears in  early, 
lymphocytes  in 
late  stages 

Lymphocytes 
considerably  in 
excess  of  poly- 
niorpho   -    nu- 
clcars 

Chiefly  polymorpho- 
nuclear cells 

Course  of 
the  dis- 
ease 

A  few  days  in  acute 
cases— commonly  2 
to 6 weeks;  chronic 
cases   may  persist 
for  several  months 

One  week  up   to 
three    or    four 
mouths 

Two    to    eight 
weeks 

A  few  days  to  two 
or  three  weeks 

Special 

features 

Erythematous,     her- 
petic,and  petechial 
rashes,      arthritic 
swellings,   cranial 
nerve  palsies 

Marked  and  per- 
sistent      head 
retraction 

Profound    mar- 
asmus        and 
emaciation 

Irregular      pulse 
and      tempera- 
ture ;      marked 
constipation  ; 
strabismus 

Convulsions,  deliri- 
um, stupor,  and 
coma 

Prognosis 

Recovery     not     un-     Usually  fatal 
common 

Invariably  fatal 

Invariably  fatal 

Part  VIII 
DISEASES   OF   THE    SPINAL   CORD 

Chaptee  I 

ANATOMY  AND  PHYSIOLOGY  OF  THE  SPINAL  CORD 

A  transverse  section  of  a  spinal  cord  shows  a  central 
mass  of  grey  matter  surrounded  by  white  substance.  A 
postero-lateral  projection  of  grey  matter  known  as  the 
posterior  horn,  and  covered  by  a  cap  of  gelatinous  substance, 
subdivides  the  white  matter  into  a  posterior  and  an  antero- 
lateral column.  The  antero-lateral  column  is  further  sub- 
divided by  the  issuing  anterior  nerve  roots  into  an  anterior 
and  a  lateral  column. 

The  grey  matter  of  the  two  halves  of  the  cord  is  connected 
by  the  posterior  or  grey  commissure,  in  which  is  situated  the 
central  canal.  The  grey  matter  contains  both  nerve  cells  and 
nerve  fibres.  The  nerve  cells  are  grouped  so  that  some 
occupy  the  entire  length  of  the  anterior  horn  throughout  the 
cord,  others  the  intermedio-lateral  horn  between  the  eighth 
cervical  and  the  second  lumbar,  and  a  third  group,  the  column 
of  Clarke,  occupies  the  base  of  the  posterior  horn  in  the  lower 
dorsal  and  upper  lumbar  regions.  Other  cells  are  scattered 
throughout  the  posterior  horn  and  intermediate  zone  of  grey 
matter.      (Fig.  87.) 

The  white  columns  of  the  cord  are  made  up  of  a  number  of 
tracts  having  different  origins  and  terminations,  and  conduct- 
ing upwards  or  downwards.  These  tracts  have  been  mainly 
differentiated  by  the  method  of  secondary  degeneration,  and 
may  be  readily  recognised  under  low  powers  of  the  microscope. 

Most  of  the  tracts  of  the  spinal  cord  have   been  already 

20 


306 


NERVOUS  DISEASES 


incidentally  described  under  the  various  systems  on  an  earlier 
page,  but  it  is  necessary  to  refer  to  them  here  in  relation  to 
the  localisation  of  function  in  the  cord. 

1.  The  posterior  columns  are  formed,  for  the  most  part, 
of  fibres  arising  in  the  posterior  root  ganglia,  which  enter  the 


I'm    Spinal  Cord 


Sevto-marginal  i  raci . 
Poetero-intemal 

milium,      I'ostero-v.t  Ivrnnl  volition. 


Posterior 
nerve  root. 

[•inferior  root 
eone  of  Bur- 
dach's  column. 
—  Lissouer's  tract. 

Direct  cere- 
bellar bract. 

Middle  root  eone  of 
Burdock's  column. 
Comma  tract, 
oot 


vomma  irai 

Anterior  roi 
zonvof  Bur- 


inch's  vol  a  in  n. 
Grossed  pyra- 
midal tract. 

Lateral  limit- 
iiui  layer. 


Enhro-spiiuil 
bract. 

Gower s's  bract. 

Antero-lateral 
ground  bundle. 


Helwea's  trad 
(spviw-oUvary). 


A  nl' trior  nerve  root. 


Mesial  cell  (  Ventral 
column.    }    Dorsal 


Anterior  spinal  artery. 


Anterior  pyra- 
midal tract. 


Cornu-vomiuissiiral  Irnvl. 
Fasciculus  sulco-m arginaUs. 
Vestibulospinal  tract. 


Fig.  87. — Diagram   showing  the  tracts,  cell   columns,  and    vascular   supply 
of  the  spinal  cord.     (Modified  from  Morris's  'Anatomy.') 


cord  at  different  levels  and  ascend  towards  the  brain.  The 
degenerations  seen  in  a  section  of  the  cervical  region  after  a 
transverse  lesion  of  the  cord  vary  according  to  the  level  of 
the  lesion.  If  the  lesion  is  low  down  (lumbar),  the  degene- 
ration is  limited  to  the  postero-internal  columns ;  if  high  up 
(cervical),  the  postero-external  columns  are  also  degenerated. 


DISEASES  OF  THE  SPINAL  CORD  307 

The  posterior  columns  also  contain  fibres  which  degenerate 
below  a  transverse  lesion.  In  the  cervical  region,  occupying 
a  position  between  the  postero-internal  and  postero-external 
tracts,  is  a  strand  of  fibres  which  degenerates  downwards 
for  a  short  distance  after  a  transverse  lesion  in  this  locality. 
This  is  the  '  comma-shaped  '  tract  of  Schultze,  formed  of 
endogenous  fibres,  probably  commissural,  between  adjacent 
segments  of  the  cervical  region.     (Fig.  88.) 


Fig.  88. — Showing  the  degenerations  descending  from  a  transverse 
lesion  in  the  cervical  region.  The  degeneration  in  the  posterior 
columns  is  the  '  comma  tract '  of  Schultze  ;  an  extensive 
degeneration  is  also  seen  in  the  antero-lateral  columns. 

Other  small  descending  tracts — the  septo-marginal  tract, 
the  oval  field,  and  the  ventral  posterior  field — are  probably 
portions  of  the  same  system  of  endogenous  fibres  at  different 
levels  of  the  cord. 

2.  The  antero-lateral  columns  contain  both  ascending  and 
descending  tracts,  some  of  which  are  of  exogenous  and  others 
of  endogenous  origin. 

(a)  Descending  Tracts.  If  we  look,  in  the  first  place,  at 
the  tracts  which  degenerate  below  a  transverse  lesion  of  the 
cord,  it  will  be  seen  that  they  occupy  both  the  posterior  and 
antero-lateral  parts  of  the  lateral  column  and  the  anterior 
column.       (Fig.  88.) 

The  crossed  pyramidal  tract  is  situated  in  front  of  the 
posterior  horn  and  is  separated  from  the  periphery,  except  in 
the  lumbo-sacral  region,  by  the  dorsal  cerebellar  tract.  If 
two  sections  of  the  cord  be  compared,  one  taken  from  below 

20  * 


308 


NERVOUS  DISEASES 


a  transverse  lesion  of  the  cord  and  the  other  from  a  case  of 
cerebral  hemiplegia,  it  will  be  seen  that  the  descending  tract 
of  degeneration  in  the  lateral  column  in  the  latter  case  is 
much  smaller  than  in  the  former.  In  the  cerebral  lesion  we 
see  the  fibres  of  the  crossed  pyramidal  tract  alone ;  in  the 
cord  lesion  another  descending  tract — the  rubro-spinal — is 
observed  in  front  of  the  pyramidal  bundle.  (Figs.  88  and  89.) 
The  rubrospinal  tract  descends  from  the  opposite  red 
nucleus  through  the  pons  and  medulla,  enters  the  cord  at 
the  decussation,  and  lies  immediately  in  front  of  the  crossed 


Fig.  89. — Showing  the  degeneration  of  one  crossed  pyramidal  tract 
resulting  from  a  cerebral  lesion  (hemiplegia). 


pyramidal  tract  and  partly  mixes  with  its  fibres.  It  can  be 
traced  into  the  mid-thoracic  region. 

The  direct  pyramidal  tract  occupies  the  marginal  area 
along  the  anterior  median  fissure,  and  mixed  with  it  are 
descending  sulco -marginal  fibres. 

Occupying  an  antero-lateral  position  in  the  periphery  of 
the  cord  is  the  antero-lateral  descending,  or  vestibulospinal 
tract,  an  exogenous  bundle  of  fibres  issuing  from  Deiters's 
nucleus.      (Fig.  87.) 

(b)  Ascending  Tracts.  A  study  of  the  tracts  which 
degenerate  in  an  ascending  direction  in  the  antero-lateral 
columns  will  reveal  the  following: — 

The  dorsal  cerebellar  tract  of  Flechsig,  which  arises  in 
the  cells  of  Clarke's  column  and  occupies  the  postero-lateral 
periphery  of  the  lateral  column  (p.  19). 

The  ventral  cerebellar  tract  of  Gowers,  occupying  the 
antero-lateral  periphery  of  the  cord  (p.  19). 


DISEASES  OF  THE  SPINAL  CORD  309 

Along  the  margin  of  the  anterior  fissure,  and  partly 
mixing  with  the  fibres  of  the  direct  pyramidal  tract,  is  the 
ascending  sulco -marginal  tract. 

A  small  triangular  bundle  of  fibres,  which  would  seem  to 
pass  to  the  inferior  olivary  body,  has  been  found  in  the  upper 
cervical  region,  and  is  known  as  Helweg's  bundle. 

Although  the  tracts  just  described  form  the  great  mass  of 
the  white  substance  of  the  spinal  cord,  those  in  immediate 
relation  to  the  grey  matter  have  received  special  designation. 
The  white  matter  lying  between  the  crossed  pyramidal  tract 
and  the  lateral  portion  of  the  grey  matter  is  known  as  the 
lateral  limiting  layer ;  and  the  white  substance  in  imme- 
diate relation  to  the  anterior  horn  is  called  the  anterior 
ground  bundle.  These  areas  of  white  matter,  with  the 
posterior  ventral  field  of  the  posterior  columns,  are  probably 
composed  of  endogenous  and  association  fibres,  linking  up 
adjacent  segments  as  well  as  those  situated  somewhat  more 
distantly. 

RELATION  OF  THE  SPINAL  SEGMENTS  TO  THE 
VERTEBRAL  SPINOUS  PROCESSES 

The  spinal  cord  is  built  up  of  a  number  of  superim- 
posed segments,  corresponding  more  or  less  approximately  to 
the  vertebral  bodies.  Each  segment  is  furnished  with  a  pair 
of  spinal  nerve  roots— anterior  and  posterior — which  enter 
and  leave  the  spinal  canal  by  the  intervertebral  foramina,  at 
varying  distances  below  the  corresponding  spinal  segments. 
As  the  spinal  cord  itself  terminates  at  the  lower  border  of  the 
first  lumbar  vertebra,  the  nerve  roots  corresponding  to  the 
lower  lumbar  and  sacral  segments  pass  vertically  downwards 
for  some  distance  within  the  canal  towards  their  foramina  of 
exit.  Here  they  form  the  cauda  equina,  in  which  the 
higher  or  lumbar  roots  lie  external  to  the  lower  or  sacral 
roots. 

It  is  therefore  apparent,  from  this  disposition,  that  the 
seat  of  a  lesion  within  a  segment  of  the  cord  is  at  a  higher 
level  in  the  vertebral  canal  than  that  indicated  by  the 
anaesthesia  of  the  corresponding  posterior  nerve  roots.  As 
the  relation  of  the  nerve  roots  to  the  vertebral  spines  and  the 
segments  of  the  spinal  cord  is  surgically  important,  a  table 


310  NERVOUS  DISEASES 

has  been  constructed  to  show  their  relative  position  throughout 
the  spinal  column. 


Table  showing   dhb   Relation   between  the   Position  oe  the 
Spinal  Segments    \\i>  the   Verteiuul  Spinous   Processus 

Lcrel  of  spinal  segment.         Vertebral  spine. 
0  1  above  C  1 

C  2  above  C  1 

C3  CI 

C4  C2 

C5  C  3 

C  G  C4 

C  7  C  5—6 

C  8  C  6—7 

D  1  C  7 

D2  Dl 

D  3  D  2 

D  4  D  2—3 

D  5  J)  3—4 

D6  D  4—5 

D  7  D  5—6 

D  8  D  6—7 

D  9  D  7—8 

D  10  D8 

D  11  D9 

T)  12  D  10 

LI  D  10—11 

L2  D  11 

L  3  D  H—12 

L  4—5  D  12 

L5-S1  D12 

Sacrum  L  1 

These  observations  show  that  in  the  upper  cervical 
and  upper  dorsal  regions,  the  nerve  roots  issue  about  one 
vertebral  body  below  the  level  of  their  corresponding  spinal 
segment  ;  that  in  the  lower  cervical  and  mid-dorsal  regions 
the  difference  amounts  to  about  a  body  and  a  half ;  ^and  in 
the  lower  dorsal  region  to  two  vertebral  bodies.     In  the  lumbar 


DISEASES  OF  THE  SPINAL  CORD  311 

region,  owing  to  the  rapid  shortening  of  the  spinal  cord, 
all  the  lumbar  segments  correspond  to  the  eleventh  and 
twelfth  dorsal,  and  the  sacral  segments  to  the  first  lumbar 
vertebra. 

Vascular  Supply  op  the  Spinal  Cord 

The  arteries  of  the  spinal  cord  are :  the  anterior  spinal 
artery,  which  passes  along  the  front  of  the  cord  and  gives 
off  a  median  branch  distributed  to  the  anterior  horns  and 
central  grey  matter  ;  and  two  smaller  posterior  spinal  arteries, 
which  pass  along  the  line  of  the  posterior  nerve  roots  and 
give  branches  into  the  posterior  horns.     (Fig.  87.) 

In  addition  to  these  main  arterial  supplies,  numerous 
small  vessels  pierce  the  periphery  of  the  cord  in  all  directions 
from  the  encircling  sheath  of  pia-arachnoid  membrane. 

The  grey  matter  is  more  richly  endowed  with  blood- 
vessels than  the  white :  a  fact  which  probably  explains  the 
greater  tendency  for  lesions  of  a  vascular  nature — such  as 
poliomyelitis  and  hemorrhage — to  occur  in  this  part  of  the 
cord. 

The  perivascular  lymphatic  spaces  communicate  with  the 
subarachnoid  space  outside  the  spinal  cord. 


LOCALISATION  OF  FUNCTION  IN  THE  SPINAL  CORD 

The  following  description  is  in  no  way  a  full  account  of 
the  functions  of  the  spinal  cord,  but  is  intended  as  a  guide  to 
the  clinical  localisation  of  lesions  within  the  cord. 

The  spinal  cord  has  the  following  functions  :— 

1.  It  conducts  motor  impulses  from  the  cerebrum  by  the 
crossed  and  direct  pyramidal  tracts. 

2.  It  conducts  sensory  impressions  to  the  cerebrum  by  the 
posterior  columns  and  certain  ascending  tracts. 

3.  It  conducts  afferent  and  efferent  cerebellar  impulses  : 
the  former  by  the  dorsal  and  ventral  spino-cerebellar  tracts, 
the  latter  by  the  vestibulo- spinal  tract. 

4.  It  contains  within  its  segments  centres  for  reflex  action. 

5.  It  contains  trophic  and  vaso-motor  centres. 

6.  It  contains  tracts  and  fibres  connecting  the  various 
segments  with  each  other. 


312  NERVOUS  DISEASES 

i.  Motor  conduction  in  the  spinal  cord 

A  lesion  of  the  pyramidal  system  in  any  part  of  its  course 
is  followed  by  degeneration  of  the  pyramidal  tracts  below 
the  level  of  the  lesion. 

The  symptoms  consequent  upon  a  lesion  of  the  pyramidal 
tracts  are  :  — 

(a)  A  spastic  paralysis  or  paresis  of  the  muscles  below  the 
lesion,  characterised  by — 

(1)  An  increase  of  the  muscle  tonus. 

(2)  Exaggeration  of  the  deep  reflexes. 

If  the  spastic  paralysis  involves  the  muscles  of  the  abdomen 
and  lower  limbs, 

(3)  Diminution  or  loss  of  the  superficial  abdominal 
and  epigastric  reflexes. 

(4)  An  alteration  in  the  character  of  the  plantar 
reflexes,  whereby  the  normal  flexion  of  the  big 
toe  is  replaced  by  extension  (p.  44). 

(b)  Impairment  of  sphincter  control — 

(1)  As  regards  the  vesical  sphincter,  either  precipitancy 
and  incontinence,  or  hesitancy  and  retention. 

(2)  As  regards  the  rectal  sphincter,  retention  of  faeces, 
except  on  the  administration  of  aperients,  when 
incontinence  is  the  rule. 

2.  Sensory  conduction  in  the  spinal  cord 

The  sensory  tracts  and  the  conduction  of  sensory  impres- 
sions in  the  spinal  cord  have  been  already  described  on  pp. 
9  to  18. 

The  following  summary  may  be  given  here  : — 

(a)  The  senses  of  discrimination  of  two  points  and  of 
passive  position  of  the  limbs  pass  up  the  homo-lateral  pos- 
terior columns,  and  cross  in  the  decussation  of  the  fillet. 

(b)  Tactile  sensibility  and  its  localisation  and  the  sense  of 
pressure  pass  up  the  homo-lateral  posterior  columns  for  an 
indefinite  distance  and  then  decussate  to  the  opposite  antero- 
lateral column. 

(c)  All  forms  of  painful  and  thermal  sensibility  decussate 
at  once  and  pass  up  by  way  of  the  opposite  antero-lateral 
columns. 


DISEASES  OF  THE  SPINAL  CORD  313 

It  is  not  possible  to  say  precisely  what  are  the  symptoms 
of  lesion  of  the  individual  sensory  columns,  but  as  far  as 
is  known  they  are  the  following : — 

Symptoms  of  lesion  of  the  posterior  columns  : 

(a)  Loss  of  the  sense  of  passive  position  of  the  limbs. 

(6)  Loss  of  cutaneous  discrimination. 

(c)  Impairment  of  light  touch,  and  cutaneous  localisation, 
dependent  upon  the  extent  or  degree  of  the  lesion. 

Symptoms  of  lesion  of  the  antero-lateral  columns  : 

(a)  Abolition  or  impairment  of  thermal  sensibility. 

(b)  Abolition  or  impairment  of  painful  sensibility  on  the 
opposite  side — the  sense  of  temperature  to  probably  a  greater 
degree  than  that  of  pain. 

3.  Cerebellar  conduction  in  the  spinal  cord 

Two  tracts,  the  dorsal  and  ventral  spino-cerebellar  tracts, 
convey  impressions  from  the  posterior  nerve  roots,  through 
the  spinal  cord,  to  the  cortex  of  the  cerebellum. 

It  is  difficult  to  state  what  the  functions  of  those  tracts 
are  individually,  and  apart  from  lesion  of  the  adjacent  tracts. 
There  are  a  few  disorders,  chiefly  of  a  degenerative  character, 
in  which  these  tracts  are  diseased,  and  in  such  cases  the  chief 
symptom  is  cerebellar  instability  (p.  478). 

Lesion  of  the  cerebellar  cortex  is  followed  by  some  degree 
of  hypotonia  of  the  homo-lateral  side.  It  might  therefore  be 
argued  that,  as  these  tracts  form  the  afferent  limb  of  the 
cerebellar  arc,  their  destruction  would  also  result  in  diminished 
muscular  tone. 

The  functions  of  the  efferent  vestibulo-spinal  tract  are 
likewise  unknown,  but  it  would  appear  probable  that  this 
tract  is  concerned  in  the  regulation  and  co-ordination  of 
muscular  movements. 

4.  Reflex  centres  in  the  spinal  cord 

Each  segment  of  the  spinal  cord  contains  reflex  centres  for 
the  corresponding  segment  of  the  body  and  limbs. 

In  its  simplest  form  reflex  action  consists  of  an  involuntary 
muscular  contraction  excited  b}T  a  peripheral  stimulation 
through  a  spinal  segment.  For  reflex  action  it  is  necessan' 
to  have  an  afferent  or  sensory  nerve  with  its  posterior  root,  a 


314  NERVOUS  DISEASES 

connexion  between  the  posterior  root  and  the  anterior  horn 
cells,  an  anterior  root  and  motor  nerve  and  a  peripheral 
muscular  mechanism. 

Normal  reflex  action  indicates  a  healthy  condition  of  the 
limbs  and  central  portion  of  the  reflex  arc  in  a  particular 
segment.  But  it  is  obvious,  from  a  consideration  of  the 
symptoms  following  lesions  of  the  conducting  paths  of  the 
spinal  cord,  that  the  reflex  centres  are  connected  both  with 
the  higher  and  the  lower  parts  of  the  nervous  system,  as 
shown  by  the  alterations  in  the  character  of  the  reflexes, 
associated  with  disease  of  these  structures.  For  example, 
the  knee  jerk  or  the  ankle  jerk  may  be  abolished  by  a 
lesion  of  the  conducting  strands  between  the  posterior  and  the 
anterior  horns ;  the  plantar  reflex,  which  in  health  consists 
mainly  of  flexion  of  the  big  and  small  toes  on  stroking  the 
sole  of  the  foot,  is  converted  into  a  movement  of  extension 
of  the  big  toe  when  the  pyramidal  tracts  are  disorganised. 
Under  a  like  condition  the  normal  contraction  of  the 
abdominal  muscles  ceases  when  the  skin  of  the  abdomen 
is  stroked,  but  the  tendon  jerks  show  varying  degrees  of 
exaggeration. 

An  examination  of  the  reflexes,  therefore,  throws  light  not 
only  upon  the  condition  of  the  spinal  segment  as  a  reflex 
centre,  but  also  upon  the  condition  of  the  conducting  paths  in 
the  brain  and  spinal  cord. 

Alterations  of  the  spinal  reflexes,  therefore,  indicate  either 
an  interference  with  the  arc  itself,  or  with  the  superior 
controlling  structures. 

1.  Tendon  jerks. 

(a)  Interruption  of  the  primary  reflex  arc  causes  impairment 
or  abolition  of  the  jerks. 

(b)  Lesion  of  the  pyramidal  system  causes  exaggeration  of 
the  jerks. 

(c)  Complete  severance  of  the  cord  causes  abolition  of  the 
jerks  below  the  lesion. 

2.  Superficial  reflexes. 

{<()  Interruption  of  the  primary  reflex  arc  causes  abolition 
of  the  reflexes. 

(6)  Lesion  of  the  pyramidal  system  leads  to  ready  exhaus- 
tion, impairment  or  abolition  of  the  reflexes. 

(c)  Complete  severance  of  the  cord  causes  their  abolition. 


DISEASES  OF  THE  SPINAL  CORD 


315 


3.  Plantar  reflexes. 

(a)  Interruption  of  the  primary  arc  causes  abolition  of  the 
reflex. 

(b)  Lesion  of  the  pyramidal  system  leads  to  an  extensor 
response. 

(c)  Complete  severance  of  the  cord  causes  abolition. 

The   substitution    of  an   extensor    for    the   normal   flexor 
response  depends  upon  some  affection  of  the  pyramidal  system. 

Segmental  localisation  of  the  principal  spinal  reflexes 


Tendon. 


Superficial. 


Biceps 

C5— 6 

Palmar 

.    C  8— D  1 

Supinator 

C  6—7 

Epigastric    . 

.    D  5— D  9 

Triceps    . 

C  6—7 

Hypogastric 

.    D  10—12 

Quadriceps 

L  3—4 

Cremasteric 

.    L  1—2 

Achilles 

S  1—2 

Plantar 

.    S  1—2 

Anal 

.    S  5 

5.  Segmental  localisation 

Each  segment  of  the  cord  contains  spinal  centres  for  the 
muscles  of  the  body  and  limbs,  and  receives  by  the  corre- 
sponding nerve  roots  sensory  impressions  from  definite  skin 
areas. 

(a)    Motor  Localisation 

Each  spinal  segment  contains  motor  cells  for  several 
muscles,  but  one  muscle,  or  a  group  of  muscles,  derives  its 
chief  supply  from  one  segment.  The  intercostal  muscles 
appear  to  be  supplied  from  a  single  segment,  but,  according 
to  Sherrington,  most  muscles  receive  nerve  fibres  from  three 
spinal  segments.  Each  spinal  segment  in  the  cervical  and 
lumbar  enlargements  presides  over  several  muscles.  A 
lesion  of  a  single  segment  weakens  the  action  of  a  number 
of  muscles,  although  the  paralysis  falls  especially  upon  one 
particular  muscle  or  group  of  muscles. 

The  symptoms  of  lesion  of  the  anterior  horns  vary 
according  to  the  individual  segment  or  segments  affected,  but 
the  following  general  features  are  characteristic  of  paralysis 
of  this  region  :  — 

Flaccid  paralysis  of  the  muscles  supplied  by  the  affected 
segment  characterised  b}' — 


Fig.  90.— Front  view  of  the  body  and  limbs  showing  the  skin  areas 
corresponding  to  bhe  spina]  segments. 


Fig.  91. — Back  view  of  the  body  and  limbs  showing  the  skin  areas 

corresponding  to  the  spinal  segments. 


318  NK  KVOUS    DISEASES 

(a)  Muscular  atrophy. 

(b)  The  reaction  of  degeneration  (p.  51). 

(c)  Loss   of   the  deep    and    superficial    reflexes    subserved 
by  the  affected  muscles. 


Table  showing  the  Principal  Muscles  am>  theih  Spinal 
Represen cations 

C  2 — 3     Deep  muscles  of  the  neck,  trapezius,  sfcerno-masfcoid. 

C  3  Levator    anguli    scapulae,    trapezius,    stomo-mastoid,    diaphragm 

Bcaleni. 

C  4  Diaphragm,  trapezius,  levator   anguli  scapulae,  soaleni,  rhomboids, 

deltoid,  spinati,  biceps,  supinator  longus. 

C  5  Deltoid,     spinati,     teres    minor,     rhomboids,     diaphragm,    biceps, 

supinator  longus,  serratus  magnus,  pectoralis  major,  brachialis 
anticus,  coraco-brachialis. 

C  6  Biceps,    coraco-brachialis,   brachialis   anticus,    supinator   longus, 

deltoid,  spinati,  teres  major,  serratus  magnus,  pectoralis  major, 
subscapularis,  pronators,  extensors  of  wrist. 

C  7  Triceps,    extensors    of  wrist    and   fingers,    pronators,    pectoralis 

major,  subscapularis,  latissimus  dorsi,  teres  major. 

C  8  Flexors     of    wrist    and     long     flexors     of    fingers,     interossei, 

lumbricales,  thenar  and  hypothenar  muscles. 

I)  1  Muscles     of     thenar     and     hypothenar     eminences,      interossei, 

lumbi'icales,  flexor  carpi  ulnaris,  oculo-pupillary  fibres. 

D  2 — 12  Intercostals,    rectus    abdominis,    serratus   posticus,    internal   and 
external  oblicpies. 

L  1 — 2     Ilio-psoas,     quadratus     lumborum,     sartorius,     cremaster,    quad- 
riceps. 

L  3  Quadriceps,   sartorius,  quadratus  lumborum,  adductores,  obturator 

externns. 

hi  Adductores,    quadriceps,   sartorius,   tensor   fasciae    femoris,   tibialis 

anticus.  extensor  communis,  extensor  liallucis. 

L  5  Tibialis  anticus,  extensor  communis  digitorum,  extensor  hallucis, 

peronei,  abductors  and  external  rotators  of  hip,  gastrocnemii, 
flexor  longus  digitorum,  hamstrings,  glutei. 

S  1  Gastrocnemii,      hamstrings,      long     flexors      of     toes,     peronei, 

abductors    and    external    rotators    of    hip,    glutei. 

S2  Glutei,      intrinsic     muscles     of    foot,     gastrocnemii,    hamstrings, 

long   flexors   of   toes. 

S3 — 5     Muscles    of    perinaeum    connected    with    defaecation,    micturition, 
erection,   ejaculation. 

Note. — Thick  type  indicates  the  principal  supply  of  each  segment. 


DISEASES  01  THE  SPINAL  CORD  319 

(h)    Sensory  Localisation 

It  has  been  demonstrated,  both  clinically  and  experi- 
mentally, that  a  lesion  which  involves  the  whole  transverse 
area  of  the  cord  in  any  one  segment,  produces  a  loss  or 
diminution  of  sensibility  below  the  level  of  the  lesion,  and 
a  slight  alteration  in  the  sensibility  in  the  areas  supplied 
by  the  segment  above.  Each  spinal  segment  receives  by  its 
posterior  nerve  roots  sensory  impressions  from  more  or  less 
definite  areas  of  the  body;  but  inasmuch  as  the  sensory 
supply  of  any  one  of  these  areas  is  provided  for,  not  by 
fibres  entering  one  posterior  root  alone,  but  partly  by  fibres 
which  enter  by  the  adjacent  superior  and  inferior  roots,  a 
complete  lesion  in  one  segment  results  in  (1)  complete  loss  of 
sensibility  below  the  level  of  the  lesion  ;  (2)  considerable,  but 
not  complete,  loss  in  the  area  supplied  chiefly  by  the  posterior 
roots  of  the  affected  segment ;  and  (3)  slight  impairment  of 
sensibility  in  the  area  supplied  by  the  posterior  roots  of  the 
segment  above. 

The  quality  of  the  sensory  loss  differs  from  that  seen  in 
lesions  of  the  peripheral  sensory  nerves  owing  to  the  readjust- 
ment of  the  grouping  of  the  various  forms  of  sensibility  in 
the  spinal  cord.  And  it  also  follows  that  partial  lesions  of 
the  spinal  cord  cause  loss  of  some  forms  of  sensibility  more 
than  of  others — dissociation  of  sensibility  according  to  the 
involvement  of  the  various  afferent  paths  within  the  cord. 

For  the  size,  shape,  and  position  of  the  several  skin  areas, 
the  reader  is  referred  to  the  annexed  charts.     (Figs.  90  and  91.) 

The  following  is  a  summary  of  the  symptoms  of  a  total 
transverse  lesion  of  the  cord  : — 

(a)  Complete  flaccid  palsy  below  the  level  of  the  lesion, 
with  wasting  of  the  muscles  and  loss  of  their  faradic 
excitability. 

(b)  Complete  abolition  of  sensation  below  the  level  of  the 
lesion,  marked  impairment  corresponding  to  the  lesion,  and 
slight  impairment  corresponding  to  the  segment  above  it. 

(c)  Abolition  of  all  the  reflexes. 

(d)  At  first  retention,  later  dribbling  of  urine  and  con- 
stipation. 

(e)  Trophic  changes  and  bed-sores. 


320  NERVOUS  DISEASES 

Chapter  II 
INTRINSIC  DISEASES  OF  THE  SPINAL  CORD 

1.  ACUTE   MYELITIS 

Although  the  term  '  myelitis  '  really  signifies  an  inflamma- 
tion of  the  spinal  marrow,  it  has  come  to  be  applied  to  a  number 
of  spinal  disorders  which  are  not  of  an  inflammatory  character. 
More  intimate  study  of  the  nature  and  pathology  of  the 
diseases  of  the  spinal  cord  has  reduced  the  number  of  different 
forms  of  so-called  myelitis,  the  great  majority  of  such  cases 
being  really  due  to  thrombotic  lesions  of  the  spinal  blood- 
vessels, arising  chiefly  from  syphilitic  endarteritis. 

The  following  are  the  disorders  to  which  the  term  'myelitis' 
has  been  applied  : — 

1.  Thrombotic  myelitis,  a  primary  condition  of  softening  of 
the  spinal  marrow  (myelomalacia)  associated  with  blocking 
of  the  spinal  blood-vessels,  and  usually  of  syphilitic  origin 
(described  on  p.  374).  A  chronic  form,  known  as  senile 
paraplegia,  is  seen  in  old  people. 

2.  Pressure  myelitis,  a  chronic  form,  attributable  to  vertebral 
disease,  meningeal  lesions,  or  to  the  pressure  of  tumours 
without  or  within  the  theca  spinalis  (described  on  p.  346). 

3.  Infective  myelitis.  This  may  be  primary,  or  secondary 
to  such  general  conditions  as  enteric  fever,  smallpox, 
dysentery,  malaria,  measles,  tonsillitis,  gonorrhoea,  and 
cystitis. 

We  propose  to  limit  the  term  acute  myelitis  to  a  somewhat 
rare  inflammatory  condition  of  the  spinal  cord. 

Infective  Myelitis — Acute  Myelitis 

Etiology.  This  may  be  primary,  or  secondary  to  such 
conditions  as  enteric  fever,  smallpox,  dysentery,  malaria, 
measles,  tonsillitis,  gonorrhoea,  and  cystitis. 

Pathology.  The  morbid  appearances  are  characteristic 
of  an  acute  infective  process  in  the  spinal  cord.  It  is 
not  more  frequent  in  the  dorsal  region,  but  may  extend, 
more  or  less,  throughout  the  whole  of  the  spinal  cord  and 


DISEASES  OF  THE  SPINAL  COED  32] 

upwards  into  the  bulb.  Both  white  and  grey  matter  share  in 
the  inflammation,  but  the  posterior  columns  are  generally 
the  seat  of  the  most  intense  changes.  The  arteries  are 
distended  and  engorged  with  blood,  and  around  them  a  marked 
proliferation  of  small  cells  is  observed  with  a  few  polymorpho- 
nuclear leucocytes.  Around  this  is  an  area  of  proliferated 
neuroglial  cells.  The  myelin  sheaths  are  swollen  and  broken 
up,  and  there  may  be  destruction  of  axis-cylinders.  The 
large  cornual  ganglion  cells  may,  or  may  not,  be  affected. 

A  similar  vascular  engorgement  and  perivascular  prolifera- 
tion is  seen  in  the  spinal  meninges. 

In  old-standing  cases  sclerosis  is  seen  in  the  affected  areas, 
and  sections  of  the  cord  show  ascending  and  descending 
degenerations. 

The  cerebro-spinal  fluid  has  been  found  to  contain  leuco- 
cytes and  diplo-bacilli.  According  to  Farquhar  Buzzard,1  the 
character  of  the  lesions  just  described  suggests  a  bacterial 
disorder  resulting  from  an  infection  of  the  lymphatic  system. 

Acute  myelitis  occurs  in  three  forms — acute  transverse 
myelitis,  acute  ascending  or  disseminated  myelitis,  and  acute 
suppurative  myelitis  or  abscess  of  the  spinal  cord. 

Acute  Transverse  Myelitis 

Symptoms.  The  onset  of  the  spinal  symptoms  is  preceded, 
or  accompanied  by,  general  symptoms  of  malaise,  fever,  and 
pain  in  the  back.  The  onset  is  usually  rapid,  without  being 
sudden,  that  is  to  say,  the  full  effect  of  the  lesion  may  take 
several  hours  to  develop.  It  is  impossible  to  lay  down  any 
rule  as  to  the  length  of  time  which  may  elapse  between  the 
onset  of  the  first  symptom  and  the  full  development  of  the 
paralysis. 

The  first  symptoms  are  usually  subjective  sensations  of 
pain,  numbness  or  burning.  When  present,  the  painful 
sensations  are  referred  to  the  upper  level  of  the  affected 
segments,  the  numbness  and  tingling  to  the  limbs  below  the 
level  of  the  lesion.  Pain  at  the  level  of  the  lesion  is  universal 
in  cases  in  which  the  lesion  is  a  meningo-myelitis,  and  as  it  is 
rare  for  an  infective  condition  to  be  limited  to  the  spinal  cord, 
pain  is  usually  present. 

1  Farquhar  Buzzard,  Brain,  1907. 

21 


322  XKUYors  DISEASES 

Numbness  or  paresthesia  of  bhe  Limbs  may  precede  weak- 
ness, but  some  degree  of  motor  paralysis  is  usually  co- 
existent.- In  the  early  stages,  reflex  spasms  of  the  extremities 
below  the  lesion  give  rise  to  pains  and  cramps,  sometimes  of 
a  severe  character.  In  other  cases,  the  motor  weakness 
develops  before  the  sensory  functions  are  involved.  This  may 
consist  first  of  a  feeling  of  heaviness  or  weight  in  the  legs, 


Fig.  92. — Shows  a  large  bed-sore  over  the  great  trochanter  in  a  case 
of  acute  myelitis. 

which  soon  gives  place  to  obvious  and  complete  motor 
paralysis. 

Loss  of  sphincter  control  may  be  an  early  symptom,  but 
in  the  acute  cases  the  sensory,  motor,  and  sphincter  functions 
usually  suffer  together  with  the  establishment  of  a  complete 
transverse  lesion  of  the  cord. 

The  clinical  picture  presented  by  such  a  case  is  as  follows  : 

1.  Symptoms  beloiv  the  level  of  the  lesion, 
{a)  Complete  flaccid  motor  paralysis. 

{]>)  Complete  loss  of  the  reflexes — superficial  and  deep. 

(c)  Complete  loss  of  all  forms  of  sensation. 

(d)  Complete  abolition  of  sphincter  control,  so  that  incon- 
tinence of  urine  and  feces  are  present. 

(e)  Trophic  changes — such  as  bed-sores.     (Fig.  92.) 

2.  Symptoms  corresponding  to  the  level  of  the  lesion,  which 
may  extend  over  two  or  more  spinal  segments, 


DISEASES  OF  THE  SPINAL  COM) 


:;:': 


(a)  Flaccid  motor  palsy  of  the  muscles  supplied  from   the 
anterior  cornual  regions. 

(6)  A  loss  of  all  forms  of  sensation  over  the  corresponding 
area.  At  the  upper  margin  sensation  may  not  be  completely 
abolished,  and  subjective  sensations  of  pain  and  constriction 
are  frequently  present  in  association 
with  objective  hyperesthesia. 

(c)  Abolition  of  the  reflexes  inner- 
vated from  the  corresponding  seg- 
ments. 

If  the  lesion  be  transverse  but 
incomplete  in  degree,  the  clinical 
picture  is  modified  chiefly  in  respect 
of  the  motor  and  reflex  signs.  The 
voluntary  power  is  lost  or  impaired 
below  the  lesion,  but  the  condition 
of  the  muscles  is  that  of  spastic 
paralysis  with  increase  of  the  my- 
otatic  irritability,  the  deep  reflexes 
are  exaggerated,  and  clonus  is  easily 
obtained. 

The  superficial  abdominal  reflexes 
are  abolished,  and  the  plantar  re- 
sponse is  of  extensor  type.  There 
may  also  be  incontinence  of  urine 
and  loss  of  expelling  power  of  the 
bladder  and  rectum. 

The  sensory  symptoms  conform 
to  those  seen  in  the  complete  trans- 
verse lesion,  in  so  far  as  the  upward 
extent  of  the  affection  of  sensibility 
is  concerned ;  but  the  degree  of 
impairment  varies  and  some  degree  of  dissociation  may  be 
observed.     (Fig.  93.) 

The  trophic  functions  are  also  disturbed,  as  in  the  com- 
plete cases. 

Prognosis.  Recovery  in  the  infective  cases  is  rare.  If 
it  occurs,  it  is  characterised  by  a  lessening  of  the  sensory  im- 
pairment and  of  the  trophic  disturbances,  by  the  development 
of  spastic  paraplegia  with  contractures — especially  of  the  flexor 
and  adductor  muscles — and  slow  return  of  voluntary  power. 

21  * 


Fig.  93. — Showing  the  dis- 
tribution of  the  sensory 
loss  in  a  case  of  acute 
meningo  -  myelitis.  The 
dark  area  corresponds 
to  the  seat  of  the  lesion 
and  represents  complete 
abolition  of  sensation  ; 
over  the  stippled  zone 
the  sensory  loss  is  in- 
complete. 


324  NERVOUS  DISK  ASKS 

The  permanent  condition  is,  as  a  rule,  that  of  persistent 
paraplegia  and  contractures. 

Treatment.  The  patient  should  be  placed  upon  a  water 
bed.  Great  care  should  be  taken  to  prevent  the  develop- 
ment of  bed-sores  ;  constant  attention  is  necessary,  all  points 
of  pressure  should  be  protected,  and  the  skin  kept  clean  and 
dry.  The  use  of  the  catheter  requires  to  be  carried  out  with 
rigid  antiseptic  precautions.  In  the  event  of  cystitis  being 
present,  the  bladder  should  be  washed  out  daily  with  a  sterile 
solution  of  boracic  acid,  and  urotropin  given  internally  every 
six  hours.  Careful  attention  should  also  be  paid  to  the 
bowels — the  rectum  being  emptied  at  a  fixed  time  each 
day  ;  the  action  of  a  mild  aperient  being  aided  if  necessary 
by  an  enema. 

Drugs  are  of  little  use  in  the  general  treatment  of  the 
disease,  although  they  may  be  of  service  in  relieving  symptoms. 
Flexor  spasms  of  the  legs  may  be  alla37ed  by  the  bromides, 
cannabis  indica,  or  veronal. 

After  the  acute  symptoms  have  passed  away,  massage  and 
passive  movements  to  prevent  contractures  and  deformities 
may  be  successfully  employed.  Spasticity  is  counteracted 
by  passive  movements,  carefully  regulated  exercises,  and  if 
necessary  extension  by  weights. 

Acute   Ascending  Myelitis 
(stn.  :  a.cute   disseminated   myelitis,  a.ccjte  a.scending 

MENINGO-MYELITIs) 

Pathology.  According  to  Buzzard,  the  pathological  changes 
consist  of  a  lepto-meningitis  and  scattered  patches  of  softening 
in  the  white  and  grey  matter.  Hemorrhages  are  not  infrequent, 
and  the  brain  may  be  hypersenric. 

Microscopically,  foci  of  acute  inflammation  are  found  either 
in  patches  or  involving  the  whole  transverse  section  of  the 
cord,  both  of  the  white  and  grey  substance.  The  vessels  are 
engorged  and  there  is  a  cellular  proliferation  in  the  adventitial 
lymphatic  sheath.  Proliferation  of  the  neuroglial  cells,  which 
are  swollen,  multi-nuclear  and  sometimes  vacuolated,  is  ob- 
served round  the  vessels.  The  nerve  fibres  may  be  swollen  or 
may  have  entirely  disappeared.     The  blood  is  not  coagulated. 


DISEASES  OF  THE  SPINAL  CORD  325 

Around  the  vessels  are  mono-nuclear  cells,  and  further  afield 
are  polyrnorpho-nuclear  leucocytes. 

Cellular  proliferation  is  also  detected  in  the  meninges. 
Amyloid  bodies  are  found  in  the  periphery  of  the  cord  and 
around  the  blood-vessels.  Degeneration  of  the  ganglion  cells 
is  in  proportion  to  the  existing  inflammation.  In  contra- 
distinction to  the  changes  found  in  acute  poliomyelitis,  the 
cells  may  be  swollen  and  chromatolytic  without  inflammatory 
exudation,  except  in  the  adjoining  white  matter. 

These  changes  may  be  due  to  many  varieties  of  bacteria, 
and  are  of  the  type  seen  in  typhoidal  myelitis,  and  in  old  cases 
of  '  urinary  paralysis.'  According  to  Buzzard,  it  is  a  bacterial 
infection  of  the  lymphatic  system  of  the  spinal  cord — a  spinal 
lymphangitis. 

Symptoms.  The  clinical  picture  presented  by  this  malady 
is  that  of  a  rapidly  ascending  motor  and  sensory  paralysis. 
The  disease  commences  suddenly,  often  with  pain  or  a  feeling 
of  constriction  round  the  abdomen  or  chest,  which  is  followed, 
some  hours  later  or  the  next  day,  by  weakness  and  numbness 
of  the  lower  limbs  and  sphincter  trouble.  During  this  period 
the  deep  reflexes  may  be  increased  and  an  extensor  plantar 
response  may  be  present.  The  weakness  develops,  often 
quite  suddenly,  into  a  complete  paraplegia,  so  that  the  condi- 
tion now  presented  is  that  of  complete  flaccid  paralysis  with 
sensory  loss  and  abolition  of  the  deep  and  superficial  reflexes, 
including  the  plantar  reflex,  below  the  level  of  the  lesion,  but 
the  electrical  reactions  remain  normal.  The  palsy  may 
spread  upwards  segment  by  segment,  or  symptoms  pointing 
to  a  focal  lesion  higher  up  the  spinal  cord  rnay  supervene. 
The  skin  is  dry,  and  there  is  great  liability  to  the  formation  of 
bed-sores. 

Although  clinically  the  symptoms  may  suggest  either  a 
steady  upward  advance  of  the  disease,  or  the  existence  of  two 
or  more  focal  segmental  lesions,  the  pathological  examination 
of  such  cases  reveals  widespread  and  disseminated  myelitis, 
which  may  be  hard  to  reconcile  with  the  clinical  appearances 
and  symptoms. 

Prognosis.  The  prognosis  is  as  a  rule  unfavourable,  the 
disease  ending  fatally,  but  cases  are  on  record  in  which 
recovery  has  taken  place.  In  those  which  do  not  die,  recovery 
may  be  relatively  complete,  the  patient  being  able  to  walk 


326 


NERVOUS  DISEASES 


with  some   spasticity  of  the  limbs    and    impaired    sphincter 
control. 

Treatment.     This  is  the  same  as  for  transverse  myelitis. 

Acute  Suppurative  Myelitis 

Suppurative  myelitis,  or  abscess  of  the  spinal  cord,  apart 
from  its  occurrence  in  the  course  of  a  suppurative  meningitis, 
is   a  rare  affection.     It  has  been  found   in  association  with 


Fig.  94. — Photograph  of  an  intraspinal  abscess,  secondary  to 
old-standing-  tuberculous  disease  of  the  spine. 

tuberculous  and  carcinomatous  disease  of  the  vertebrae,  which 
have  led  to  evascularisation  and  necrosis  of  the  corresponding 
spinal  segments,  and  therefore  favoured  the  attacks  of  bacilli 
from  the  bladder  or  elsewhere.  It  may  also  occur  as  a 
primary  condition.  The  pus  in  these  cases  spreads  upwards 
and  downwards  from  the  original  lesion  and  occupies  a  posi- 
tion chiefly  in  the  anterior  regions  of  the  posterior  columns 
and  horns.  In  some  places  definite  signs  of  an  abscess  wall 
maybe  discerned,  but  the  spread  of  the  pus  seems  to  be  along 
the  lines  of  least  resistance,  causing  secondary  destruction  of 
the  adjacent  white  matter.     (Fig.  94.) 

The  abscess  cavity  occupies  a  position  similar  to  the  lesion 
in  syringom}Telia,  and  to  hemorrhage  within  the  spinal  cord. 

Symptoms.  When  primary,  the  onset  is  similar  to  that  of 
acute  transverse  myelitis.  When  secondary  to  tuberculous 
or  carcinomatous  disease  of  the  vertebrae,  the  onset  may  be 
subacute. 


DISEASES  OF  THE  SPINAL  CORD  S27 

As  a  general  rule,  it  is  impossible  to  distinguish  this  con- 
dition from  acute  transverse  myelitis ;  but  abscess  has  been 
diagnosed  during  life  in  the  secondary  cases  by  the  presence 
of  dissociated  sensibility  and  the  signs  of  an  intramedullary 
lesion. 

The  prognosis  is  most  unfavourable. 

Treatment.     This  is  the  same  as  for  transverse  myelitis. 


2.    CAVITIES  IN   THE  SPINAL  CORD 

There  are  four  conditions  in  which  cavity  formation  is 
found  in  the  spinal  cord. 

(a)  Hydromyelia,  or  distension  of  the  central  canal, 
unaccompanied  by  structural  changes  other  than  those  due 
to  compression  of  adjacent  tissues.  No  symptoms  may  be 
present,  but  its  importance  lies  in  the  fact  that  injuries  to 
the  spine  may  lead  to  hemorrhage  into  the  cavity. 

(b)  Gliosis,  or  new  formation,  occurring  in  the  central 
grey  matter,  within  which  cavity  formation  occurs  as  a  result 
of  degenerative  changes. 

(c)  Cystic  degeneration  of  gliomatous  and  sarcomatous 
tumours  within  the  spinal  cord  (p.  336). 

(d)  Tuberculous,  vascular,  or  inflammatory  lesions,  which 
may  result  in  softening  and  cavity  formation  (p.  374). 

(e)  Syphilitic  pachymeningitis. 

As  the  two  last  conditions  are  described  under  their 
respective  headings,  the  condition  known  as  spinal  gliosis 
will  receive  consideration  only  in  this  place. 

Syringomyelia  or  Spinal  Gliosis 

Etiology.  Syringomyelia  occurs  more  commonly  in  men 
than  in  women  ;  the  most  frequent  period  for  its  commence- 
ment is  between  twelve  and  thirty  years  of  age.  There  is  no 
predisposing  cause ;  but  in  many  cases  the  history  of  a  spina 
bifida  may  be  obtained,  or  a  cicatrix  detected,  on  examination 
of  the  back.  Injuries  to  or  falls  on  the  lower  part  of  the 
spine  have  been  cited  as  exciting  causes,  but  conclusive 
evidence  on  this  point  is  lacking. 

Pathology.  The  essential  feature  of  this  disease  is  the 
occurrence    of   a    gliosis    with    cavity    formation.       This   is 


328  NERVOUS  DISEASES 

probably  fcbe  result  of  a  proliferation  of  the  ependymal  cells 
and  tissues  surrounding  the  central  canal.  Retrograde 
changes  occur  in  the  centre  of  the  new  growth,  resulting  in 
the  formation  of  cavities,  which  may  contain  a  serous  or 
gelatinous  fluid.  The  cavity  is  not  lined  with  ependymal 
cells,  except  in  the  rare  cases  in  which  it  opens  into  the 
central  canal.  The  gliosis  is  characterised  by  a  profusion  of 
glial  fibres,  amongst  which  is  scattered  a  relatively  small 
number  of  neuroglial  cells. 

The  growth  occupies  the  grey  matter,  and  tends  to  in- 
vade the  anterior  horn,  the  anterior  portion  of  the  posterior 
columns,  and  to  extend  as  a  fissure  along  the  posterior  horn. 

Examination  by  the  naked  eye  shows  the  cord  at  the  level 
of  the  cavities  either  increased  or  diminished  in  size.  When 
increased,  the  cord  feels  to  the  touch  like  an  india-rubber 
tube.  In  transverse  section  the  walls  of  the  cavity  present  the 
appearance  of  a  glistening  thick  membrane,  and  ascending 
and  descending  degenerations  may  be  visible  in  the  columns 
of  the  cord. 

The  portions  of  the  cord  chiefly  affected  are  the  cervical 
and  upper  dorsal  regions,  the  lumbar  region,  and  sometimes 
the  medulla  oblongata  (syringo-bulbia). 

The  view  that  this  condition  is  congenital  is  supported 
by  those  cases  in  which  the  remains  of  a  spina  bifida,  or 
meningocoele,  are  found  in  association  with  the  cavity  for- 
mation and  when  the  cavity  is  lined  with  ependymal  cells. 
In  many  cases,  on  the  other  hand,  there  is  no  evidence  of  a 
congenital  origin,  and  the  condition  ought  to  be  regarded  as 
a  neuroglial  overgrowth. 

Symptomatology.  The  symptoms  of  the  disease  vary 
according  to  the  situation  and  extent  of  the  lesion.  If  the 
cervical  and  upper  dorsal  portions  of  the  cord  are  alone 
affected,  the  local  symptoms  will  be  referred  to  the  upper 
limbs  and  to  the  cervical  sympathetic ;  if,  on  the  other 
hand,  the  condition  extends  upwards  to  the  medulla,  bulbar 
symptoms  will  be  present. 

Syringomyelia  produces  motor  and  sensory  symptoms  in 
one  or  both  of  two  ways. 

(a)  By  interfering  with  incoming  sensory  and  outgoing 
motor  fibres  through  lesion  of  the  central  grey  matter 
of  the  affected  segment  or   segments.     In  the  early  stages 


DISEASES  OF  THE  SPINAL  CORD  329 

these  may  be  the  only  symptoms,  and  consist  of  segmental 
loss  of  either  thermal  sensibility,  thermal  and  painful  sensi- 
bilities, or  later,  of  all  forms  of  sensation  in  the  corresponding 
root  areas,  which  may  or  may  not  be  associated  with  segmental 
muscular  atrophies.  The  afferent  sensory  fibres,  especially 
those  which  decussate  immediately  after  their  entrance  into 
the  cord,  are  apt  to  be  involved  by  the  growth,  which  extends 
posteriorly  from  the  central  grey  matter  into  or  along  the 
posterior  horn.  This  explains  the  dissociation  of  sensation 
which  is  found  corresponding  to  the  level  of  the  lesion,  as 
the  fibres  which  subserve  painful  and  temperature  sensibilities 
cross  at  once,  while  those  for  tactile  sensation  pass  mainly 
up  the  same  side  (p.  17). 

(b)  The  growth  of  the  lesion  may  induce  a  further  series 
of  symptoms  by  pressure  upon  the  long  ascending  and 
descending  conduction  strands.  The  symptoms  of  the  later 
stages  are,  therefore,  impairment  of  all  forms  of  sensibility 
and  spastic  paralysis  below  the  level  of  the  lesion,  of  either 
unilateral  or  bilateral  distribution. 

It  is  possible  that  if  the  lesion  does  not  extend  eccentrically 
so  as  to  interfere  with  the  functions  of  the  grey  matter  and 
adjacent  commissural  fibres,  a  cavity  of  considerable  length 
may  exist  without  giving  rise  to  symptoms.  This  explains 
the  finding  of  syringomyelic  cavities  at  autopsies  upon  cases 
in  which,  during  life,  no  symptoms  had  been  detected. 

In  the  majority  of  cases  the  clinical  picture  presents  the 
following  features  : — - 

1.  At  the  level  of  the  lesion. 

(a)  A  dissociation  of  thermal  and  painful  from  tactile 
sensibility. 

(b)  Progressive  muscular  wasting. 

(c)  Vaso-motor  and  trophic  changes. 

2.  Below  the  level  of  the  lesion.  In  the  late  stages  loss  of 
sensation  and  spastic  paralysis. 

The  disease  shows  itself  primarily  by  disturbances  of 
sensibility,  by  the  occurrence  of  painless  burns,  or  by  trophic 
alterations.  As  the  lesion  commonly  involves  the  cervical 
region  of  the  cord,  these  changes  are  observed  in  the  upper 
limbs.  Pains  radiating  down  the  arm  or  up  the  neck  may  be 
an  early  symptom,  but  numbness  and  a  feeling  of  deadness  are 
the  most  frequent   subjective   sensations.     These  symptoms 


330 


NERVOUS  DISEASES 


may  be  accompanied,  or  shortly  followed,  by  some  degree  of 
motor  weakness  in  the  arms  or  shoulders,  and  later  by 
atrophic  paralysis.  On  the  one  hand,  this  condition  may 
remain  stationary  for  varying  periods.  On  the  other,  a 
progressive  or  intermittent  increase  of  the  symptoms  may 
supervene,     with    signs     indicating     interference    with     the 


Figs.  Ho  \m.  '.Hi.-  Illustrate  paralysis  of  the  cervical  sympathetic  nerve  on  the 
left  side.  Tlic  partial  ptosis,  enophthalmos,  and  contracted  pupil  are 
well  seen. 


functions  of  the  tracts  of  the  spinal  cord  above  or  below 
the  level  of  the  focal  symptoms. 

Optic  nerves.  Optic  atrophy  and  contraction  of  the 
visual  fields  have  been  observed,  but  are  rare. 

Oculo-motor  nerves.  Nystagmoid  jerkings,  or  nystagmus 
on  extreme  lateral  deviation,  are  present  in  a  large  number 
of  cases.  Ocular  palsies,  especially  of  the  sixth  nerve,  are 
also  found.  The  pupillary  light-reaction  is  retained,  but 
inequality  of  the  pupils  may  be  observed  from  paralysis  of 
the  sympathetic  nerve. 

Fifth  nerve.  The  sensory  division  is  occasionally  affected, 
more  especially  on   one   side,  either   with  pains   and  hyper- 


DISEASES  OF  THE  SPINAL  CORD  331 

sesthesia,  or  with  diminution  or  loss  of  all  forms  of  sensibility  ; 
in  some  cases,  however,  tactile  sensibility  is  retained.  Trophic 
disturbances  and  motor  paralysis  are  rare. 

Seventh  nerve.  Facial  palsy  of  the  peripheral  type  may  be 
present,  with  wasting  and  atrophy  of  the  facial  muscles. 

The  eighth  nerve  is  rarely  involved. 


Fig.  £.7. — Illustrates  muscular  atrophy  in  a  case  of  syringomyelia. 

Bulbar  nerves.  Unilateral  or  bilateral  paralysis  of  the 
tongue,  vocal  cords,  pharynx,  and  palate  are  commonly 
observed. 

Cervical  sympathetic.  This  is  more  often  affected  than 
the  other  nerves  by  reason  of  its  situation  in  the  cervical 
region.  Occasionally  irritative  phenomena,  but  more  fre- 
quently paralytic  symptoms,  are  observed — narrowing  of  the 
palpebral  fissure,  enophthalmos,  and  a  contracted  pupil,  which 
does  not  dilate  on  stimulation  of  the  skin  of  the  neck  or  to 
cocain,  but  reacts  to  light  and  accommodation.  (Figs.  95 
and  96.) 

The  order  of  implication  of  the  cranial  nerves  in 
syringomyelia  is  from  below  upwards,  and  is  due  to  the 
spread  of  the  disease  from  the  spinal  cord  into  the  bulb, 
whereby  the  bulbar  nerves  are  affected  first,  and  the  sixth 
pair  are  involved  before  the  third  nerves. 

Motor  system.  The  earliest  motor  symptom  is  a  pr one- 
ness to  fatigue,  often  accompanied  by  a  tired  aching  pain, 
generally  in  the  upper  extremities.  Later  on,  wasting  of  the 
muscles  with  fibrillation  is  noted.  In  the  majority  of  cases 
this  commences  first  of  all  in  the  small  muscles  of  the 
hands — interossei,  thenar,  and  hypothenar  groups — eventually 


332 


NERVOUS  DISEASES 


spreading  into  the  forearm,  arm,  and  shoulder.  In  a  smaller 
proportion  of  cases,  the  scapulohumeral  muscles  are  the  first 
affected.  This  is  seen  by  inability  to  raise  the  arms,  which 
hang  flail-like  by  the  sides.  The  wasting  is  bilateral,  but 
often  predominates  on  one  side.  There  is  also  a  type  of  the 
disease  in  which  the  lower  limbs  participate  in  the  muscular 
wasting.  In  these  cases  the  syringomyelia  either  commences 
in  the  lumbar  enlargement  or  secondarily  involves  it. 

The  muscular  wasting 
does  not  necessarily  spread 
in  a  definite  order  through- 
out the  limb,  but  may  miss 
portions ;  for  example,  it 
may  proceed  from  the 
hand  to  the  shoulder, 
leaving  out  the  arm.  The 
paralysis  is  often  more 
marked  than  would  be  sug- 
gested by  the  atrophic  state 
of  the  muscles.  Spastic 
paralysis  from  interference 
with  the  pyramidal  system 
may  be  bilateral  or  uni- 
lateral ;  and  if  severe  in 
degree  is  always  accom- 
panied by  sensory  changes. 
Sensory  system.  The 
disturbances  of  sensibility, 
as  already  explained,  are  of 
two  kinds — one,  segmental,  corresponding  to  the  segment  or 
segments  of  the  cord  involved ;  the  other,  involving  all 
forms  of  sensation  in  the  body  and  limbs  below  the  level  of 
the  lesion. 

1.  Local  changes.  In  the  earliest  stages  subjective  sensa- 
tions of  heat  and  cold,  with  or  without  coexistent  sensations 
of  a  painful  character,  are  present,  but  are  shortly  replaced 
by  objective  thermo-anresthesia,  moderate  degrees  of  tempera- 
ture being  felt  merely  as  sensations  of  contact.  In  early 
cases  the  loss  of  temperature  sensibility  is  limited  to  moderate 
degrees  of  heat  and  cold,  but  where  there  is  complete  loss  of 
the  extremes  of  temperature,  the  moderate  degrees  are  also 


Fid.  US.  Showing  wasting  of  the 
small  muscles  of  the  hand  aiid 
ulnar  side  of  forearm. 


DISEASES  OF  THE  SPINAL  COKI) 


abolished.  Loss  of  sensation  to  heat  or  cold  is  not  necessarily 
coterminous,  as  either  the  one  or  the  other  may  be  abolished 
over  a  greater  area. 

The  loss  of  the  sensibility  to  painful  impressions  may  be 
preceded,  in  rare  cases,  by  subjective  sensations  of  a  painful 
character,  associated  with  cutaneous  hyperalgesia.  Analgesia, 
both  superficial  and  deep,  is  the  next  most  common  sensory 
defect,  and  is  so  profound  that  burns,  cuts,  and  wounds  may 
be  inflicted  without  the  patient  being 
aware  of  them. 

Analgesia  is  not  necessarily  present 
as  the  earliest  symptom,  nor  is  it 
always  coterminous  with  the  area 
of  thermo-ansesthesia.  Once  estab- 
lished it  persists,  although  varia- 
tions within  small  limits  may  occur. 
Its  distribution  is  usually  segmental, 
yet  in  many  cases  this  is  lost,  or  so 
modified  as  to  resemble  the  '  glove ' 
analgesia  of  hysteria. 

In  the  early  stages  and  in  typical 
cases  tactile  sensibility  is  unimpaired ; 
but  in  certain  cases,  especially  in  an 
advanced  stage,  impairment  or  loss  of 
tactile  sensibility  may  be  observed, 
usually  over  areas  not  corresponding 
to  those  of  thermal  and  painful 
anaesthesia. 

The  local  sensory  changes,  although 
not  necessarily  confined  to  the  upper 
limbs  and  upper  part  of  the  chest 
and  face,  are  more  commonly  found 
in  these  localities,  owing  to  the 
position  of  the  lesion  within  the  cervical  region  of  the 
spinal  cord.      (Fig.  99.) 

2.  Distal  changes.  Impaired  sensibility  may  also  be 
present  below  the  level  of  the  lesion,  from  interference  with 
the  conducting  tracts.  If  the  lesion  is  bilateral,  impairment 
of  all  forms  is  found ;  but  more  commonly,  owing  to  one  side 
of  the  spinal  cord  being  more  involved  than  the  other,  the 
sensory   loss   assumes   the   features   of  the   Brown- Sequard 


Fig.  99.— Chart  of  the  sen- 
sory loss  in  a  case  of 
syringomyelia.  The  ver- 
tical lines  indicate  the 
area  of  thermal  and 
painful  loss ;  the  hori- 
zontal lines  indicate  the 
area  of  tactile  loss. 


334  NERVOl'S   DISK  ASKS 

'symptom-complex,'  painful  and  thermal  sensibilities   being 

lost  on  tin.'  side  opposite  the  lesion,  whereas  tactile  sensibility 
may  be  impaired  on  the  same  side  (p.  15). 

Reflexes.  The  reflexes  of  the  affected  segments  are 
abolished  ;  but  below  the  lesion  the  deep  reflexes  are  increased, 
the  epigastric  or  abdominal  abolished,  and  the  plantars  extensor 
in  type. 


Fig.  100. — Shows  tin-  pincer  hand  ('main  en      Pig.   101.- — Shows   the  suc- 
pince')  of  syringomyelia.  culent    hand     ('main 

succmlente '). 

The  sphincters  are  rarely  affected  in  the  cervical  cases, 
but  in  cases  with  a  lumbar  lesion  incontinence  is  an  early 
symptom . 

Trophic  disturbances.  The  cutaneous  changes  are  acute 
hyperemia,  redness,  and  local  elevation  of  temperature,  per- 
sisting for  several  hours  or  days.  Passive  hyperemia  is  a 
late  phenomenon,  and  often  appears  over  bony  prominences, 
and  is  due  to  mechanical  obstruction  to  the  circulation. 
Another  form  of  passive  hyperemia  is  characterised  by  slight 
swelling  of  a  bluish  tint  and  reduction  of  local  temperature. 
Secretory  changes  are  found  in  increase  or  decrease  of  sweat 
secretion,  the  former  more  commonly  in  the  earlier  stages, 
and  generally  over  the  areas  of  impaired  sensation.  A  vesci- 
cular  eruption  in  certain  nerve  areas,  lasting  for  a  few  days, 
without  pain  or  irritation,  and  often  confined  to  the  analgesic 
zones,  has  been  observed  sufficiently  frequently  to  deserve 
mention. 

The  succulent  hand  ('main  succulente')  depends  upon  vaso- 


DISEASES  OE  THE  SPINAL  CORD  335 

motor  disturbance.  The  hand  is  swollen,  especially  over  the 
dorsum,  the  skin  is  of  a  normal  colour,  pits  on  pressure,  but 
soon  recovers.  The  local  temperature  is  raised.  A  similar 
condition  is  found  in  hemiplegics,  where  the  hand  is  kept  in 
a  dependent  position.     (Figs.  100  and  101.) 

Painless    whitlows    (Mor van's    disease)    appear    upon    the 


Fig.  102. — Illustrates  painless  whitlows  (Morgan's  disease)  in  a 
case  of  syringomyelia. 

fingers.  These  may  be  an  early  feature  of  the  disease,  but  in 
many  cases  they  are  absent  throughout.      (Fig.  102.) 

Arthropathies,  similar  to  those  seen  in  tabes  dorsalis,  may 
occur,  usually  in  the  upper  limbs,  affecting  chiefly  the  shoulder, 
elbow,  and  inter-phalangeal  joints.  They  are  often  the  first 
symptoms  of  the  disease.  Spontaneous  fractures  are  also 
liable  to  occur. 

Scoliosis,  or  kypho-scoliosis  of  the  spinal  column,  has  been 
ascribed  to  articulatory  changes,  but  is  more  probably  due 
to  weakness  of  the  spinal  musculature.  It  may  be  an  early 
or  a  late  symptom.  It  is  always  found  in  the  upper  dorsal 
region. 

Diagnosis.  In  typical  and  well  defined  cases  of  syringo- 
myelia the  diagnosis  is  easy,  as  there  is  no  other  condition  in 
which  an  association  of  muscular  atrophy  with  dissociation  of 


336  XHKVOl's  DISEASES 

sensibility  as  above  described  occurs.  As  the  sensory  changes 
are  usually  the  earliest  signs,  there  is  little  likelihood 
of  the  malady  being  confused  with  amyotrophic  lateral 
sclerosis. 

The  two  affections  which  are  most  likely  to  be  confused 
with  syringomyelia  are  :  (1)  spinal  tumour  with  root 
Bymptoms,  and  (2)  cervical  rib. 

In  spinal  tumours,  the  local  paralyses  correspond  to  the 
affected  nerve  roots  ;  the  sensory  loss  is  of  the  peripheral  type 
and  usually  unilateral ;  the  pressure  symptoms  develop  first 
upon  the  same  side  as  the  root  symptoms. 

In  cases  of  cervical  rib,  the  palsy  is  usually  unilateral  and 
confined  to  certain  root  areas  ;  the  sensory  loss  is  of  the 
peripheral  type  ;  there  are  no  associated  spinal  symptoms,  and 
an  X-ray  examination  shows  an  additional  rib. 

Leprous  neuritis,  in  the  early  stages,  may  present  a  close 
resemblance  to  syringomyelia,  owing  to  the  patchy  character 
of  the  anaesthesia,  and  muscular  atrophy.  The  previous 
history,  thickening  of  the  nerves  and  the  absence  of  evidence 
of  interference  with  the  functions  of  the  spinal  cord,  are  the 
determining  points. 

Prognosis.  The  disease  is  progressive,  but  may  persist  for 
many  years.  Eecovery  is  unknown,  although  arrest  of  the 
symptoms  sometimes  takes  place. 

Treatment.  This  is  conducted  on  general  principles ; 
massage,  faradisation,  and  the  treatment  of  complications  as 
they  arise.  Special  care  must  be  taken  to  avoid  injury  or 
burning  over  the  anaesthetic  areas. 

3.   INTRAMEDULLARY   TUMOURS 

The  common  forms  of  tumour  within  the  spinal  cord  are 
glioma,  sarcoma,  tubercle,  and  gumma.  Hydatids  have 
occasionally  been  found. 

Gliomata,  the  commonest  form  of  new  growth  within  the 
brain,  are  rarely  met  with  in  the  spinal  cord.  These  tumours 
tend  to  break  down  and  form  one  of  the  varieties  of  cavity 
formation  within  the  cord.  This  may  be  primary,  analogous 
to  that  seen  in  the  cerebellum,  or  it  may  be  secondary  to 
hemorrhage  within  the  tumour.  It  is  distinguished  from 
syringomyelia  by  the  presence  of  numerous  neuroglial  cells, 


DISEASES  OF  THE  SPINAL  CORD  337 

with  few  neuroglial  fibres,  and  by  the  preservation  in  an  intact 
state  of  the  central  canal  and  its  ependymal  lining.  These 
growths  primarily  originate  within  the  grey  matter,  and 
commonly  spread  into  the  white  substance. 

Sarcomata  are  either  primary  within  the  cord,  or  second- 
ary to  growths  in  the  cerebrum,  cerebellum,  spinal  roots,  or 
meninges.     Meningeal  sarcoma  may  invade  the  spinal  cord 


Pig.  103. — Showing  a  large  intramedullary  tumour  in  the  cervical 
region  of  the  spinal  cord. 

along  the  pial  vessels,  the  vessels  of  the  central  grey  sub- 
stance, or  along  the  deep  fissures.  In  two  cases  in  which 
a  primary  growth  invaded  the  fourth  ventricle,  secondary 
deposits  were  found  growing  from  the  interior  of  the  central 
canal.  Usually  the  cells  are  of  the  small  or  large  round 
type.  It  is  rare  for  such  growths  to  attain  any  size  without 
degenerative  changes  and  cavit}7  formation  occurring  in  then- 
central  parts. 

Tuberculous  tumours  occur  in  association  with  general 
tuberculous  infection  of  the  meninges,  but  have  also  been 
found  as  solitary  growths  similar  to  those  occurring  in  other 
parts  of  the  central  nervous  system. 

Gummata  are  always  found  in  connexion  with  the  pia- 
araclmoid  membrane  or  spinal  blood-vessels. 

22 


338 


XEHVOl'S   DISEASES 


The  following  are  the  ages  at  which  the  different  types  of 
intramedullary  tumour  are  most  common  :  — 


Under  12  years 
12  to  40  years 
Over  40  years 


Tubercle,  sarcoma,  glioma. 
Glioma,  tubercle,  gumma. 
Gumma,  sarcoma. 


Symptomatology.     Intramedullary    tumours    give  rise   to 
symptoms  depending  upon  their  situation  rather  than  their 


Fig.  10-i. — Photograph  of  the  spinal  cord  showing  cavity  forma- 
tion in  association  wiili  spinal  gliosis. 


nature.  They  form  a  connecting  link  clinically  between 
syringomyelia  and  the  intradural  form  of  extramedullar 
growths.  Pure  intramedullary  tumours,  or  those  which  have 
not  extended  from  the  meninges,  are  rare,  and  present  a 
symptomatology  in  all  respects  similar  to  that  described  under 
syringomyelia  (p.  328). 

Intramedullary  tumours  growing  from  the  meninges  and 
secondarily  involving  the  cord  present  symptoms  corre- 
sponding to  those  found  both  in  syringomyelia  and  extra- 
medullary  tumours,  but  with  the  important  clinical  distinction 
that  the  early  symptoms  are  those  of  an  extramedullary 
growth  (p.  350). 

These  may  be  for  a  time  the  only  symptoms,  but  the 
physical  examination  reveals  signs  which  cannot  be  explained 


DISEASES  OF  THE  SPINAL  CORD  339 

entirely  by  the  existence  of  an  extramedullar  growth,  and 
point  to  the  presence  of  an  intramedullary  lesion. 

The  earliest  signs  are  found  in  the  realm  of  sensation. 

1.  Local  segmental  dissociation  of  sensation  (loss  of  pain- 
ful and  thermal,  with  retention  of  tactile  sensibilities). 

2.  The  occurrence  of  the  Brown-Sequard  '  symptom- 
complex  '  below  the  level  of  the  lesion  (loss  of  painful  and 
thermal  sensibility  on  the  side  opposite  the  motor  paralysis). 


Pig.  105. — Two  charts  showing  the  sensory  impairment  in  a  case 
of  intramedullary  tumour  of  the  cervical  region.  The 
black  area  (A)  is  the  region  of  complete  anassthesia  and 
corresponds  to  C  3  and  4  segments.  B  is  an  area  of 
incomplete  loss,  and  0  a  region  of  impaired  thermal 
sensation. 

The  motor  signs  are  segmental  paralyses  of  an  atrophic 
character,  in  which  muscular  atrophy  is  in  excess  of  motor 
weakness. 

Amongst  other  symptoms,  the  early  onset  of  sphincter 
trouble,  the  early  occurrence  of  an  extensor  plantar  response, 
and  in  a  general  way  signs  of  an  extensive  unilateral  lesion, 
point  to  an  intramedullary  new  growth. 

With  the  extension  of  the  growth  signs  of  bilateral  spastic 
paraplegia  and  sensory  loss  below  the  level  of  the  lesion 
become  apparent,  and  the  local  segmental  motor  and  sensory 

■i-i  * 


340  NERVOUS  DISEASES 

changes,  which  were  at  the  outset  confined  to  one  side,  become 
bilateral. 

Diagnosis.  The  diagnosis  has-  to  be  made  mainly  from 
syringomyelia.  In  this  condition,  as  alreadj7  described  on 
p.  328,  the  disease  runs  a  more  prolonged  course,  the 
characteristic  dissociation  of  sensation  is  well  marked  and 
is  accompanied  by  muscular  wasting.  Painless  whitlows, 
arthropathies,  trophic  disturbances,  and  kypho-scoliosis  are 
also  outstanding  features  of  syringomyelia. 

Prognosis.  The  disease  progresses  to  complete  paraplegia. 
It  varies  in  duration,  but  lasts  about  two  or  three  years  in 
average  cases.  Death  occurs  from  cystitis,  bed-sores,  or  other 
complications. 

Treatment.  The  treatment  consists  in  the  alleviation  of 
symptoms  as  they  arise,  and  the  prevention  of  cystitis  and 
bed-sores.  A  course  of  anti-syphilitic  treatment  may  be 
prescribed  in  doubtful  cases,  and  in  some  cases  an  exploratory 
laminectomy  is  justifiable. 

4.  ELEMATOMYELIA— <  SPINAL  APOPLEXY' 

Hemorrhage  into  the  spinal  cord  is  rare  as  a  primary 
condition.  It  may  occur  in  consequence  of  trauma  following 
a  direct  injury  or  a  sudden  jar  of  the  vertebral  column,  such 
as  results  from  a  fall  upon  the  feet  or  the  lower  part  of  the 
spine.  It  has  also  been  observed  to  follow  a  severe  exertion, 
such  as  lifting  a  heavy  w7eight.  We  have  also  known  it  happen 
in  consequence  of  diving  into  water  from  a  height. 

Secondary  hemorrhage  may  take  place  into  softened  areas 
resulting  from  myelitis,  or  into  tumours  within  the  spinal 
marrow.  In  two  cases  personally  examined,  hemorrhage  into 
the  cord  occurred  in  consequence  of  a  tuberculous  abscess 
outside  the  dura  mater. 

It  is  important  also  to  note  the  occasional  presence  of 
spinal  hemorrhage  in  syringomyelia,  both  in  those  cases 
which  have  presented  the  clinical  signs  of  this  disease,  as  well 
as  in  those  in  whom  no  clinical  evidence  of  syringomyelia  had 
been  previously  observed,  but  where  an  autopsy  showed  the 
existence  of  cavity  formation.  It  is,  therefore,  not  unlikely 
that  in  the  cases  of  spinal  hemorrhage  attributed  to  exertion, 
the   underlying  cause  is  a  pre-existing   cavity.     It  has  also 


DISEASES  OF  THE  SPINAL  CORD  341 

been  seen  in  morbid  blood  states — such  as  pernicious  ansemia. 
Spinal  hemorrhage  has  a  special  tendency  to  affect  the  grey 
matter  of  the  cervical  and  lumbar  enlargements. 

Symptoms.  The  onset  is  usually  sudden,  with  a  rapid 
development  of  symptoms  to  their  maximal  extent.  In  other 
cases  the  occurrence  of  spinal  hemorrhage  leads  to  a  more 
ingravescent  development  of  symptoms,  whose  maximum  is 
not   reached   for"  a*! few   hour3.     Consciousness   is   not   lost. 


Fig.  108. —  Photograph  showing  the  position  of  the  arms  in  an  old-standing 
case  of  haematomyelia  of  the  cervical  region. 

Pain  at  the  seat  of  the  lesion,  or  referred  to  the  affected  root 
areas,  is  sometimes  a  transient  symptom  ;  and  the  tempera- 
ture, normal  at  first,  may  be  slightly  raised  for  a  few  days. 

The  symptoms  in  a  severe  case  are  complete  motor  and 
sensory  paralysis  to  the  level  of  the  lesion.  The  motor  palsy 
is  at  first  of  a  flaccid  type,  the  deep  reflexes  are  abolished, 
and  there  is  retention  of  urine. 

The  first  sign  of  improvement,  which  may  occur  in  from 
one  to  three  weeks,  is  a  restitution  of  the  tactile'and  pressure 
sensibilities,  and  the  sense  of  position  of  the  limbs.  During 
this  period  the  deep  reflexes  gradually  return,  the  flaccidpalsy 
by  degrees  gives  place  to  spasticity,  which  is  accompanied  by 


342 


XKKYors   DISEASES 


flexor  or  extensor  spasm  of  the  limbs,  and  an  extensor  plantar 

response. 

Voluntary    movement   returns    slowly,    and    may   be   first 

shown  in  flexion  of  the  toes.     The  degree  of  motor  power  is 

impaired  by  the  presence  of  spasm  and  the  development  of 

spastic  contractures.  The  retention 
of  urine  gradually  gives  place  to 
reflex  incontinence,  and  constipation 
becomes  marked.  Trophic  sores  may 
develop  during  the  first  week,  and 
frequently  prove  an  obstinate  compli- 
cation. 

In  less  severe  cases  the  ultimate 
recovery  may  be  nearly  complete, 
the  patient  being  able  to  walk  with 
a  spastic  gait,  but  with  good 
sphincter  control,  and  perhaps  some 
permanent  loss  of  painful  and  thermal 
sensibilities  below  the  level  of  the 
lesion. 

Atrophic  paralyses  and  sensory 
changes  corresponding  to  the  level 
of  the  lesion  may  become  permanent. 
In  some  cases  the  hemorrhage 
is  limited  to  one-half  of  the  spinal 
cord,  and  gives  rise  to  the  Brown - 
Sequard  symptom,  already  described 
p.  15  and  Fig.  107). 

The  local  segmental  symptoms 
vary  according  to  the  region  of  the 
cord  into  which  the  hemorrhage 
has  occurred.  If  in  the  lower  cer- 
vical region,  oculo-pupillary  changes, 
atrophy   of  the  intrinsic   muscles   of 

the   hand,  and    sensory  impairment  along  the  ulnar  border 

are  observed. 

If  in  the  lumbo- sacral  region,  atrophic  palsy  of  the  lower 

limbs,  loss  of  the  deep  reflexes,  permanent  sphincter  trouble, 

and  obstinate  bed-sores  are  found. 

Differential  diagnosis.     The  differential  diagnosis  has  to 

be  made  from  : — 


Fig.    L07.     Chari     showing 

I  lie  sensory  loss  in  a  east' 
of  unilateral  haemato- 
nivelia.  A,  area  of  com- 
plete loss  to  all  forms 
of  sensation.  B,  region 
of  painful  and  thermal 
ansesl  hesia.  ( '.  area  of 
thermal  and  painful 
anaesthesia  on  the  side 
opposite  the  lesion.  D, 
impairment  of  the  sense 
of  ]iassi\  o  mo\  ement  of 
toes  and  foot. 


DISEASES  OF  THE  SPINAL  CORD 


1.  Acute  poliomyelitis,  by  the  absence  of  constitutional 
symptoms,  the  sudden  onset  following  trauma  or  exertion, 
the  well-marked  sensory  changes,  and  the  localisation  of  the 
symptoms  to  a  segmental  level.  We  have  seen  one  case 
in  which  a  hemorrhage  was  strictly  limited  to  the  anterior 
horn  on  one  side  in  the  cervical  enlargement,  resulting  in 
a  flaccid  palsy  of  the  arm  without  sensory  change. 

2.  Acute  myelitis,  by  the  suddenness  of  the  onset, 
the     absence     of     constitutional 

symptoms,  a  history  of  trauma, 
and  the  dissociation  of  sensation 
either  in  the  earlier  or  the  later 
stages. 

3.  Meningeal  hemorrhage,  by 
the  lesser  degree  and  extent  of  the 
pain,  the  more  rapid  and  more 
complete  paralysis,  and  the  sen- 
sory dissociation.  In  traumatic 
cases  the  use  of  the  X-rays  may 
be  of  value  as  demonstrating  a 
fracture-dislocation,  and  a  lumbar 
puncture  will  show  the  presence  of 
blood  in  the  cerebro-spinal  fluid. 

Prognosis.  Death  is  rarely 
the  result  of  the  primary  lesion, 
but  may  ensue  from  septic  absorp- 
tion from  bed-sores  or  cystitis  and 
from  pyelonephritis.  In  all  but 
the  most  severe  cases  some  degree 

of  recovery  may  be  anticipated.  Atrophic  paralysis  from 
local  destruction  of  nerve  elements  at  the  seat  of  the 
hemorrhage  tends  to  remain  permanent. 

In  forming  a  prognosis  as  to  the  recovery  from  the 
symptoms  after  lesion  of  the  conducting  tracts  of  the  cord, 
the  following  points  require  consideration. 

The  points  in  favour  of  a  satisfactory  prognosis  are  :— 

(a)  The  early  return  of  sensation,  although  painful  and 
thermal  sensibilities  may  be  permanently  abolished. 

(b)  The  early  recovery  from  the  flaccid  palsy,  with  return 
of  the  deep  reflexes. 

(c)  The  healing  of  trophic  sores  within  six  weeks. 


Fig.  108.  —  Shows  the 
'  preacher's  hand '  in  case 
of  haeinatomYelia. 


344  XKKYOUS  MSKASKS 

\<h  The  early  return  of  voluntary  control  over  the  bladder. 

(e)  The  return  of  voluntary  power  in  the  limhs  within 
two  or  three  months. 

Of  unfavourable  omen  are  permanent  loss  of  all  forms 
of  sensation,  the  retention  of  complete  flaccidity  and  the 
development  of  severe  spastic  contractures,  the  continuance 
of  bed-sores  and  incontinence  of  urine,  and  the  absence  of 
any  voluntary  power  after  three  months. 

Treatment.  Rest  is  essential.  The  patient  should  prefer- 
ably not  be  allowed  to  lie  upon  his  back,  but  rather  on  his 
side  upon  a  water-bed.  The  urine  should  be  drawn  off  by  a 
catheter.  Great  care  should  be  taken  to  keep  the  patient  dry, 
especially  over  the  points  of  pressure. 

Ergot  has  been  recommended  in  the  early  stages. 

After  the  acute  stage  has  passed,  passive  movements  and 
massage  of  the  paralysed  limbs  may  be  given.  Later  on,  the 
spasticity  has  to  be  combated  by  hot-air  baths,  followed  by 
passive  movements  and  massage,  especially  to  the  extensors. 
If  there  is  a  tendency  to  contracture  extension  may  be 
applied,  care  being  taken  to  avoid  injury  to  the  skin.  For 
the  atrophic  paralyses,  massage  and  galvano-faraclism  should 
be  given. 

If  the  reflex  spasms  are  severe  a  combination  of  trional 
and  phenacetin,  or  a  mixture  of  the  bromides  and  cannabis 
indica,  may  be  prescribed. 

o.  CAISSON  DISEASE  AND  DIVER'S   PARALYSIS 

This  disease  occurs  in  those  who  work  under  high  atmo- 
spheric pressures,  not  during  the  period  in  which  they  are 
subjected  to  the  pressure,  but  after  their  return  to  ordinary 
atmospheric  conditions. 

The  factor  necessary  to  its  production  is  exposure  to 
compressed  air  followed  by  a  sudden  diminution  of  atmo- 
spheric pressure.  The  longer  the  exposure  and  the  more 
rapid  the  return  to  normal  conditions,  the  more  likely  are 
nervous  symptoms  to  ensue.  It  has  also  been  noted  that 
persons  of  temperate  habits  suffer  less  than  those  addicted 
to  alcohol. 

During  the  period  when  the  worker  is  exposed  to  the  high 
pressure,  the  blood  becomes  surcharged  with  gases,  and  if  the 


DISEASES  OK  THE  SPINAL  COED  545 

pressure  is  diminished  too  rapidly,  bubbles  of  gas  escape  from 
the  small  blood-vessels  into  the  nervous  system  and  destroy 
the  surrounding  nerve  tissues.  Air  emboli  also  may  form 
in  the  capillaries  and  result  in  minute  softenings.  There  has 
been  much  discussion  as  to  the  nature  of  the  gases,  some 
writers  holding  that  nitrogen,  others  that  oxygen  and  car- 
bonic acid  gas  are  the  main  constituents. 

Pathology.  A  microscopic  examination  reveals  changes 
in  the  dorsal  region  of  the  spinal  cord,  more  especially  in  the 
white  matter  of  the  posterior  and  lateral  columns.  The 
changes  consist  of  small  fissures,  surrounded  by  zones  of 
parenchymatous  myelitis,  disintegration  of  myeline,  and  swell- 
ing of  the  axis  cylinders.  Small  areas  of  localised  softening 
may  also  be  observed.  The  grey  matter,  nerve  roots,  and 
meninges  are  not  affected.     Hemorrhages  are  rare. 

Symptoms.  These  vary  considerably,  both  as  regards  in- 
tensity and  character.  In  the  main  they  are  those  of  a  spinal 
lesion,  but  in  other  cases  well-marked  cerebral  symptoms  may 
also  be  present. 

The  cerebral  symptoms  consist  of  headache,  vertigo, 
throbbing,  and  deafness,  often  accompanied  by  nausea  and 
vomiting.  In  some  cases  a  transient  coma  occurs,  more  rarely 
monoplegic  or  hemiplegic  symptoms  persist. 

The  spinal  symptoms  are  more  general  and  affect  chiefly 
the  sensory  system,  but  in  severe  cases  the  motor  is  also 
affected.  In  slight  cases  tingling,  numbness  in  the  trunk, 
legs,  and  arms,  and  sometimes  severe  pains  referred  to  the 
joints  are  described.  In  association  with  the  subjective 
sensory  symptoms,  complaint  is  made  of  heaviness  and 
weakness  of  the  legs.  In  severe  cases  the  picture  is  that 
of  a  transverse  lesion  of  the  cord,  with  complete  motor, 
sensory,  and  reflex  paralyses. 

On  examination  the  objective  sensory  changes  may  be 
slight  or  wanting,  but  in  more  pronounced  cases  a  diminution 
of  all  forms  of  sensibility,  irregular  and  patchy  in  distribu- 
tion, is  observed. 

In  the  motor  system  the  weakness  varies  from  slight 
paresis  with  increased  tendon  jerks  to  complete  paralysis  with 
loss  of  the  deep  reflexes.  In  other  cases  some  inco-ordina- 
tion  of  movements  without  much  loss  of  power  may  be 
detected. 


346  NERVOUS  DISEASES 

The  diagnosis  presents  no  difficulty,  the  symptoms  coming 
on  more  or  less  rapidly,  either  immediately  or  within  a  short 
period  after  entering  the  normal  atmosphere. 

The  prognosis  is  good,  relatively  few  fatal  cases  being- 
recorded.     Recovery  from  the  symptoms  may  he  complete. 

Treatment.  This  is  mainly  preventive,  and  consists  in 
making  the  return  from  the  highly  compressed  air  as  slow  as 
possible.  The  active  treatment  consists  in  applying  recom- 
pression, the  patient  being  again  put  under  increased  pres- 
sures, which  are  gradually  diminished.  If  the  pain  be  severe, 
morphia  ma}7  be  necessary.  Should  permanent  changes  have 
occurred  in  the  nervous  system,  the  treatment  is  that  of  an 
acute  myelitis. 


Chapter    III 

DISEASES   AND    INJURIES    GIVING   RISE   TO 
COMPRESSION  OE  THE   SPINAL  CORD 

A  well-recognised  clinical  picture  is  presented  by  cases  in 
which  the  symptoms  of  progressive  paraplegia  are  due  to 
pressure  upon  the  spinal  cord,  although  both  the  mode  of 
onset  of  the  symptoms  and  their  subsequent  development 
and  course  may  vary  considerably.  It  is  obvious  that  if  the 
primary  disease  affects  the  structures  surrounding  the  spinal 
cord  before  actually  exerting  pressure  upon  it,  the  initial 
symptoms  may  be  vague  and  uncertain.  An  illustration  of 
this  is  seen  in  the  indefinite  local  pain  and  discomfort  in 
the  back  in  spinal  caries  and  malignant  disease  of  the 
vertebrae. 

On  the  other  hand,  symptoms  directly  due  to  pressure 
upon  the  spinal  cord,  or  its  roots,  tend  to  persist  and  to 
progress.  Although,  as  a  rule,  the  onset  of  symptoms  is 
gradual,  a  more  or  less  rapid  development  is  not  uncommon. 
In  such  cases  the  onset  is  not  so  rapid  as  in  myelitis,  and 
the  early  symptoms  are  less  extensive.  For  example,  in 
myelitis,  paraplegia  may  develop  within  a  few  hours,  and 
exhibit  motor,  sensory,  and    reflex  changes  below  the  level 


DISEASES  OF  THE  SPINAL  CORD  347 

of  the  lesion.  In  the  compression  cases,  complaint  may  be 
made  of  sudden  loss  of  power  in  one  leg,  with  perhaps 
numbness  in  both  legs  ;  or  a  sudden  pain  is  referred  to  a 
certain  root  area,  with  signs  of  motor  weakness  in  the  leg  on 
the  same  side.  In  the  majority  of  cases,  however,  a  slow, 
progressively  developing  paraplegia  is  the  rule. 

If  compression  is  exerted  upon  the  anterior  aspect  of 
the  cord,  the  primary  symptoms  are  motor,  and  consist  of 
spastic  paraplegia  below  the  level  of  compression,  followed 
later  by  interference  with  sphincter  control  and  the  develop- 
ment of  sensory  phenomena.  If,  on  the  other  hand,  pressure 
is  exerted  on  the  posterior  aspect,  sensory  symptoms  (sub- 
jective and  objective)  accompany,  and  may  precede,  the  motor 
symptoms.  Frequently,  however,  the  compressing  body  is 
situated  laterally,  and  involves  anterior  or  posterior  roots. 
In  such  cases,  if  a  posterior  root,  or  roots,  be  involved,  pain 
referred  to  that  root,  followed  by  anaesthesia  over  its  distri- 
bution, is  the  first  and  most  obtrusive  symptom.  This  is 
followed  by  a  motor  weakness,  most  marked  on  the  side  of  the 
affected  posterior  root,  and  sensory  symptoms  on  both  sides 
below  the  root  level,  but  more  intense  on  the  opposite  side. 
Symptoms  due  to  pressure  upon  the  anterior  roots  are  of  late 
onset,  owing  to  the  resistance  offered  by  these  roots  to  the 
effects  of  pressure,  and  to  the  absence  of  subjective  signs 
over  their  distribution.  In  compression  lesions  of  the  cauda 
equina,  atrophic  muscular  changes  form  early  and  important 
clinical  signs. 

The  following  are  the  conditions  in  which  symptoms  of 
compression  paraplegia  may  be  found: — 

1.  Tumours  of  the  spinal  membranes— 

(a)  Extrathecal ;  and 

(b)  Intrathecal. 

2.  Spinal  caries — tuberculous  disease  of  the  vertebra?. 

3.  Malignant  disease  of  the  vertebral  bodies. 

4.  Traumatic  lesions  of  the  vertebral  column. 

The  symptoms  of  intramedullary  tumours  and  of  syringo- 
myelia are  elsewhere  described  (pp.  328  and  338). 


348 


xKinors  D1SKASKS 


I.  TUMOURS  OF  THE  SPINAL   MEMBRANES 

These  tumours  are  divided  primarily  into  two  main  groups  : 
(,i)  Those  outside  the  thera  spinalis — Extradural. 
(6)  Those  inside  the  theea  spinalis     Intradural. 

They  may  arise  in  or  grow  from  the  nerve  roots,  the 
spinal  membranes,  the  extradural  connective  tissues,  the 
periosteum,  or  the  bones  of  the  vertebral  column.  The)'  vary 
in  character  and  position,  and  in  the  frequency  of  their 
occurrence  in  different  localities. 

The  following  table,  founded  upon  the  records  of  the  Queen 
Square  Hospital,  shows  the  varieties  of  tumour  found  within 
the  spinal  canal  and  the  frequency  of  their  occurrence. 


Tumour.               Intradural. 

Extradural. 

Total 

Sarcoma 

2 

5 

7 

Endothelioma 

5 

1 

0 

Echinococcus 

(Hydatid) 
Gumma 

0 
3 

5 
0 

5 
3 

Eibro-myxosarcoma 

3 

1 

4 

Fibro-sarcoma 

4 

0 

4 

Fibro-myxoma 

2 

2 

4 

Cyst 

Myxoma 

Psammoma 

2 
1 
1 

0 
0 
0 

2 
1 

1 

Fibroma 

1 

0 

1 

Angeioma 

1 

0 

1 

Cylindroma 
Uncertain 

1 

2 

0 
0 

1 

2 

28 


14 


J2 


According  to  the  table  just  given,  the  intradural  tumours 
are  twice  as  frequent  as  the  extradural.  The  extradural 
sarcomata  spring  mainly  from  the  periosteum  of  the  verte- 
bra. The  endotheliomata  attached  to  the  nerve  roots 
and  the  membranes  are  chiefly  intrathecal.  Hydatids 
seem  to  spread  from  the  deep  muscular  tissues  of  the  back, 


DISEASES  OF  THE  SPINAL  CORD 


349 


or  the  subpleural  tissues,  and  enter  the  spinal  canal  by 
erosion  of  the  lamina;.  Other  common  forms  of  intradural 
growth  are  the  slow-growing  fibro-  and  myxo-sarcomata. 


Pig.  109. 


Fig.  110. 


Figs.  109  and  110  are  photographs  of  extramedullar^,  but  intrathecal,  tumours 
pressing  upon  the  spinal  cord.  Fig.  110  is  a  tumour  involving  more 
especially  the  cauda  equina. 

The  following  table  shows  the  localities  most  favoured  by 
extramedullar  tumours  and  the  frequency  of  their  occurrence 
in  a  total  of  twenty-one  operated  cases  : — 


:r>0  NERVOUS  DISEASES 


Locality. 

Intradural. 

/•:. 

rt  radii  nil. 

Tot 

Upper  cervical  . 

3 

0 

3 

Cervico-dorsal  . 

1 

0 

1 

Dorsal 

2 

7 

9 

Dorso-lumbar   . 

2 

1 

3 

Lumbo-sacral   . 

1 

2 

3 

Cauda  equina    . 

1 

1 

2 

10  11  21 

Meningeal  tumours  may  occur  at  any  age,  but  they  are 
rarely  seen  under  ten  or  over  fifty.  The  most  common 
time  for  their  occurrence  is  between  the  ages  of  thirty  and 
fifty,  during  which  period  twelve  out  of  twenty-one  cases  were 
observed  and  treated. 

Males  are  slightly  more  affected  than  females,  the  propor- 
tion being  twelve  of  the  former  to  nine  of  the  latter. 

Symptoms.  In  the  majority  of  cases  of  tumour  pressing 
upon  the  spinal  cord,  jjremonitory  symptoms  are  found  prior 
to  the  onset  of  definite  paralysis  of  the  limbs.  Pain  in 
one  form  or  another  is  the  most  common.  Of  the  varieties 
described,  pain  and  stiffness  in  the  back,  aching  in  the 
limbs,  pain  of  a  rheumatic  character  about  the  neck  and 
shoulders,  and  occasionally  sharp  shooting  pains  in  the  leg 
or  about  the  hip  joints,  may  be  mentioned.  The  position  of 
pain  is  dependent  upon  the  site  of  tumour  within  the  spinal 
canal.  In  some  cases  the  pain  is  of  a  dull,  aching  character, 
referred  to  the  shoulder  or  the  breast  when  the  growth  is 
in  the  upper  dorsal  region.  In  other  cases  the  pain  is 
definitely  referred  to  the  distribution  of  a  particular  root  or 
roots,  and  may  be  accompanied  by  hyperesthesia  over  the 
skin  area  supplied  by  the  root. 

We  have  found  the  intense,  agonising,  burning  pains, 
described  by  some  writers  as  characteristic  of  spinal  tumour, 
to  be  extremely  rare,  such  pains  being  present  in  only  three 
out  of  twenty-one  cases  of  meningeal  tumour  submitted 
to  operation.  The  pain  as  above  described  is  unassociated 
with  any  local  tenderness  along  the  nerves,  but  when  referred 
to  the  spine  may  be  accompanied  by  some  tenderness  on  per- 
cussion, and  may  occasionally  be  increased  on  movements  of 
the  back  or  neck. 


DISEASES  OF  THE  SPINAL  CORD  351 

In  other  cases,  even  from  the  commencement,  pain  is 
entirely  absent,  the  earliest  symptom  being  weakness  upon 
one  side. 

The  premonitory  symptoms  may  persist  for  an  indefinite 
time  before  signs  of  compression  of  the  cord  make  their 
appearance.  This  period  may  vary  from  a  few  weeks  up  to 
several  years.  During  this  period  the  cause  of  the  pain  is 
quite  uncertain,  and  various  diagnoses,  such  as  rheumatism, 
intercostal  neuralgia,  lumbago,  appendicitis,  or  hip-joint 
disease,  may  be  made  according  to  the  seat  of  the  pain. 

Symptoms  of  compression  of  the  cord,  however,  sooner  or 
later  make  their  appearance.  They  are  seen  in  varying 
degrees  of  motor  and  sensory  loss.  The  motor  phenomena 
are  usually  those  of  spastic  paralysis  below  the  level  of  the 
lesion.  Generally  one  limb,  that  on  the  side  of  the  lesion,  is 
affected  before  the  other.  Complaint  is  made  of '  a  giving  way 
of  the  leg,'  and  on  examination  the  distal  portion  is  found 
more  affected  than  the  proximal.  As  time  goes  on  both  lower 
limbs  become  paralysed. 

During  the  development  of  the  motor  paralysis  the  onset 
of  two  further  symptoms  is  observed — flexor  spasms  of  the 
legs  and  impairment  of  sphincter  control.  The  former  occur 
early,  first  in  the  limb  homolateral  to  the  tumour,  and  later 
in  both  limbs.  The  latter  only  occurs  early,  when  the  tumour 
presses  upon  the  lumbo-sacral  enlargement  or  its  nerve  roots. 
In  tumours  situated  above  the  lumbar  enlargement,  the  onset 
of  sphincterweakness  is  synchronous  with  the  development  of 
bilateral  motor  weakness. 

The  symptoms  of  compression  of  the  sensory  fibres  are 
both  subjective  and  objective  in  character.  The  former  con- 
sist mainly  of  numbness  corresponding  to  the  paralysed  limb 
or  limbs,  and  appear  either  with  the  onset  of  the  paralysis  or 
shortly  afterwards.  The  objective  sensory  s}rmptoms  are 
late  in  onset  and  consist  of  impairment,  or  loss,  of  all  forms 
of  sensation  below  the  level  of  the  lesion.  By  this  feature 
meningeal  tumours  may  usually  be  distinguished  from  those 
within  the  spinal  cord,  in  which  dissociation  of  sensibility 
is  an  early  and  characteristic  symptom.  The  delimitation 
of  the  extreme  upper  level  of  sensory  change  is  one  of  the 
most  important  points  in  the  examination  of  these  cases,  as 
by  it  the  position  of  the  tumour  is  definitely  localised.     In 


352 


XKPvVOrs    DISEASES 


lesions  limited  to  the  cauda  equina  the  sensory  loss  presents 
a  definite  root  distribution  (p.  120). 

The  motor  and  sensory  phenomena  usually  coincide  in 
distribution ;  but  not  always,  as  one  side  of  the  cord  is 
frequently  more  compressed  than  the  other.  Owing,  there- 
fore, to  the  greater  unilateral  com- 
pression, Brown- Sequard's  '  symptom 
complex  '  may  be  observed,  in  which 
the  motor  palsy  is  more  pronounced 
upon  one  side,  and  the  sensory  loss 
upon  the  other. 

Beflexes.  The  deep  are  exagger- 
ated, the  superficial  abdominal  are 
impaired  or  lost,  and  the  plantar  ex- 
tensor in  type.  In  the  early  stages 
these  changes  arc  confined  to  the 
side  of  the  lesion. 

In  addition  to  the  symptoms  of 
compression  of  the  cord,  local 
symptoms  due  to  compression  of 
the  nerve  roots  at  the  level  of  the 
lesion  are  found,  viz.  atrophic  motor 
paralyses,  hyperesthesia  or  loss  of 
sensation  in  the  affected  roots. 

The  spinal  column.  Prominence 
of  the  spines  over  a  meningeal 
growth  is  rare,  but  is  a  common 
feature  should  the  tumour  originate 
in,  or  secondarily  involve  the  vertebral 
bodies.  Tenderness  on  percussion 
over  the  seat  of  the  tumour  is  some- 
times detected.  There  is  rarely  any  limitation  of  movement, 
nor  does  movement  usually  give  rise  to  local  pain,  except  in 
cases  of  tumour  high  up  in  the  cervical  region. 

Regional  diagnosis.  From  the  standpoint  of  sympto- 
matology there  are  four  chief  localities  in  which  tumours  are 
found : — 

1.  Cervico-dorsal  (lower  cervical  and  upper  dorsal)  region. 

2.  Mid-dorsal  region. 

3.  Lumbo-sacral  region. 

4.  The  cauda  equina. 


Fig.  111. — Chart  showing 
the  sensory  impairment 
in  a  case  of  tumour 
pressing  upon  1  be  spina] 
cord  at  the  level  of  the 
fourth  dorsal  segment. 
Below  the  lesion  the 
sensory  impairment  is 
more  marked  on  i  he  lefl 
side  (p.  351). 


DISEASES  OF  THE  SPINAL  CORD  353 

1.  Gervico-dorsal  region.  At  the  level  of  the  lesion  motor 
weakness  of  the  forearm  and  hand,  either  spastic  in  character 
from  pressure  upon  the  cord,  or  atrophic  and  flaccid  from 
pressure  upon  the  emerging  roots,  and  symptoms  of  irritation 
or  paralysis  of  the  cervical  sympathetic  will  be  seen.  Sensory 
impairment  will  be  present  along  the  ulnar  side  of  the  hand 
and  arm  as  far  up  as  a  line  joining  the  anterior  and  upper 
margins  of  the  axillary  folds. 

Below  the  lesion  paralysis  with  spasticity  and  impairment 
or  loss  of  sensation  are  found.  The  deep  reflexes  are  increased, 
the  plantar s  are  extensor,  and  the  superficial  abdominal 
diminished  or  lost.  Sphincter  impairment  only  occurs  when 
motor  weakness  is  bilateral.  Priapism  may  occasionally  be 
observed.  These  signs  may  not  be  equally  pronounced  on  the 
two  sides,  more  especially  in  the  upper  limbs,  while  below 
the  lesion  the  motor  and  sensory  impairment  may  approach 
the  Brown-Sequard  type  of  paralysis. 

2.  The  dorsal  and  upper  lumbar  regions.  The  motor 
palsy  is  of  the  spastic  paraplegic  type,  and  the  sensory  loss 
corresponds  to  the  level  of  the  lesion.  The  reflexes  below  the 
lesion  and  the  state  of  the  sphincters  are  as  described  above. 

3.  The  lumbosacral  region.  Here  a  meningeal  tumour 
is  not  so  likely  to  involve  several  roots  as  one  situated 
within  the  theca.  The  symptoms,  therefore,  are  those  of 
compression  paraplegia  with  increase  in  the  deep  reflexes  of 
the  lower  limbs,  and  extensor  plantar  responses  with  sphincter 
impairment.  The  abdominal  and  epigastric  reflexes  are 
retained. 

4.  The  cauda  equina.  For  an  account  of  the  symptoms 
of  lesion  of  the  cauda  equina,  the  reader  is  referred  to 
p.  120. 

Diagnosis.  The  following  points  would  favour  the  diag- 
nosis of  a  tumour  pressing  upon  the  spinal  cord  : 

(a)  A  history  of  pain,  sometimes  of  a  dull,  aching  character, 
at  other  times  sharp  and  shooting  in  the  distribution  of 
particular  nerve  roots,  for  an  indefinite  period  preceding  the 
onset  of  paraplegia. 

(b)  A  progressive  paraplegia,  usually  affecting  one  lower 
limb  before  the  other,  or  in  cervical  cases  the  arm  and  leg 
upon  the  side  on  which  root  symptoms  were  first  present. 

(c)  Motor  and  sensory  paralysis  below  a  fixed  segmental 

23 


:;:»!  NERVOUS  DISEASES 

level,  all  forms  of  sensation  being  impaired  or  lost,  but  with  a 
tendency  to  the  Brown-Sequard  distribution. 

((/)  Absence  of  sphincter  weakness,  or  only  slight  impair- 
ment, until  the  paraplegia  is  well  developed. 

(e)  The  absence  of  signs  of  caries  of  the  spine,  or  of 
malignant  disease  of  the  vertebra?. 

The  diagnosis  between  an  extraihecal  and  an  intrathecal 
tumour  is  more  of  academic  than  practical  interest,  and 
in  many  cases  is  impossible,  but  the  following  are  the  chief 
indications  which  help  in  the  diagnosis.  If  intrathecal,  the 
tumour  usually  gives  rise  to  root  symptoms  earhy,  and  these 
are  followed  more  or  less  rapidly  by  symptoms  of  compression 
of  one  side  of  the  spinal  cord,  some  time  elapsing  before  the 
establishment  of  signs  of  compression  of  the  whole  cord. 

In  extrathecal  cases  the  compression  of  the  cord  takes  place 
more  gradually,  as  the  spinal  cord  may  be  displaced  before 
being  compressed.  When  compression  occurs,  the  symptoms 
are  those  of  compression  of  the  whole  cord,  without  any 
definite  stage  in  which  the  symptoms  point  to  compression 
only  of  one  side. 

It  is  in  cases  of  tumour  of  the  lumbo-sacral  region  that 
the  difference  is  most  marked.  Here  an  intrathecal  tumour 
presses  on  several  nerve  roots  as  they  pass  to  their  point  of 
emergence.  Hence  the  symptomatology  is  that  of  a  root 
lesion  affecting  the  higher  lumbar  areas,  viz.  atrophic 
paralysis  with  root  anaesthesia  and  signs  of  compression 
paralysis  in  the  parts  below.  Thus  there  are  loss  of  the  knee 
jerks,  atrophy  of  the  thigh  muscles  with  increase  of  the  ankle 
jerks,  and  extensor  responses  and  sphincter  impairment. 
The  sensory  loss  is  that  of  a  root  lesion  in  the  upper  lumbar 
areas  with  compression  anaesthesia  below  this  level. 

The  differential  diagnosis  has  to  be  made  from 

1.  Intramedullary  tumour,  by  the  absence  of  root  pains, 
the  presence  of  muscular  atrophy,  and  the  dissociation  of 
tactile  from  painful  and  thermal  sensibility. 

2.  Disease  of  the  bones  of  the  spine  I  tuberculous  and 
malignant ) . 

3.  Aneurism  of  the  aorta,  eroding  the  vertebral  bodies  and 
compressing  the  spinal  cord. 

Treatment.  As  soon  as  the  diagnosis  of  meningeal 
tumour  has  been  established,  operation  for  its  removal  ought 


DISEASES  OF  THE  SPINAL  COKD  355 

to  be  undertaken.  The  spinal  segments  corresponding  to  the 
upper  limit  of  the  sensory  impairment  should  be  exposed,  and 
the  upper  and  lower  limits  of  the  tumour  laid  bare.  Should 
no  tumour  be  found,  and  the  cord  not  pulsate,  the  lamina; 
higher  up  ought  to  be  removed. 

Pulsation  in  the  spinal  cord  after  removal  of  the  tumour  is 
a  favourable  prognostic  sign. 

Kecovery  after  operation  for  removal  of  the  growth  may  be 
interfered  with  by  (a)  the  malignant  character  of  the  growth 
and  the  impossibility  of  its  complete  extirpation  ;  (b)  irre- 
parable destruction  of  the  spinal  cord,  owing  to  excessive  and 
long-continued  pressure  ;  and  (c)  the  formation  of  adhesions 
and  cicatricial  bands. 


2.  CARIES    OF    THE    SPINE 

Tuberculous  disease  of  the  spine  may  occur  at  any  age ; 
although  more  common  in  children,  it  is  not  at  all  infrequent 
during  adult  life.  In  most  cases  it  is  associated  with 
tubercle  elsewhere  in  the  bones,  joints,  lungs,  or  lymphatic 
glands. 

In  a  small  number  of  cases  it  develops  apparently  as  a 
primary  condition. 

The  onset  is  usually  insidious  and  without  a  definite 
exciting  cause,  but  cases  are  met  with  in  which  the  first 
symptoms  have  been  noticed  after  an  injury  or  strain  to 
the  spine. 

Pathology.  The  original  pathological  lesion  is  a  tuber- 
culous osteomyelitis  or  periostitis.  This  commences  either 
within  or  on  the  anterior,  superior,  or  posterior  surface  of  the 
body  of  one  or  more  of  the  spinal  vertebrae,  most  commonly 
of  the  dorsal  region,  and  leads  to  the  formation  of  a  soft 
caseous  tuberculous  focus  within  the  vertebral  body.  In 
consequence,  a  collapse  of  the  affected  bone  occurs  and  the 
corresponding  spinous  process  becomes  prominent.  The 
adjacent  vertebrae,  above  and  below,  are  therefore  approximated 
towards  each  other,  and  their  spines  take  part  in  the  formation 
of  the  antero-posterior  deformity,  which  is  characteristic  of 
this  condition.  The  tuberculous  process  may,  if  small  and 
localised,   undergo  calcareous    degeneration ;    in  other  cases 

23  * 


356  NERVOUS  DISEASES 

the  body  of  the  vertebra  may  disintegrate  and  be  partly 
absorbed  with  the  formation  of  granulation  tissue ;  in  others, 
abscess  formation  ensues,  and,  more  rarely,  a  sequestrum  of 
bone  is  left  with  the  development  of  a  sinus.  The  condition 
known  as  '  caries  sicca '  is  brought  about  by  the  absorption 
of  the  granulation  tissue,  leaving  only  carious  bone.  The 
reparative  process  consists  of  ankylosis  of  adjacent  vertebrae. 

The  spinal  cord  may  be  involved  in  several  ways,  of  which 
the  following  may  be  mentioned  : — 

1.  Cases  in  which  there  is  no  obvious  deformity  of  the 
spine  or  local  tenderness  on  pressure,  but  in  which  the  spinal 
marrow  is  compressed  by  a  small  abscess,  or,  more  commonly, 
granulation  tissue  extending  into  the  neural  canal.  In  these 
cases  the  symptoms  are  more  motor  than  sensory. 

2.  Cases  in  which  there  is  no  abscess  within  the  neural 
canal,  but,  owing  to  the  collapse  of  the  vertebral  bodies, 
an  angular  curvature  is  produced.  This  may  result  in  the 
stretching  and  consequent  compression  of  the  spinal  cord 
over  the  projecting  posterior  surface  of  the  vertebral  body. 

3.  Cases  in  which  a  combination  of  tuberculous  tissue  — 
abscess  or  granulation  tissue— and  deformity  causes  pressure 
upon  the  spinal  cord.  In  both  these  groups  the  symptoms 
are  largely  motor  in  excess  of  sensory. 

It  is  more  probable,  however,  that  the  effect  upon  the 
spinal  marrow  is  an  indirect  one,  and  is  brought  about 
through  circulatory  disturbances,  arising  from  the  pressure  of 
tuberculous  matter  and  thickening  of  the  dura  mater,  upon 
the  lymphatics  and  blood-vessels  of  the  cord  in  the  immediate 
neighbourhood  of  the  lesion.  In  consequence  of  this  a 
localised  cedema  of  the  cord  is  induced  (Schmaus). 

It  is  probable  also  that  a  state  of  local  evascularisation, 
or  ischemic  necrosis,  may  be  the  immediate  effect  of  the 
obstruction  to  the  blood  supply  of  the  spinal  cord,  and  the 
cause  of  the  permanent  paralysis  in  old- standing  cases  of 
the  disease. 

In  these  cases  extensive  thickening  of  the  dura  mater,  and 
adhesion  between  it,  the  pia  arachnoid  and  the  spinal  cord, 
are  observed.  This  pachymeningitis  is  not  necessarily  limited 
to  the  anterior  surface  of  the  cord,  but  is  found  all  round  it, 
compressing  it  and  interfering  with  the  vascular  supply. 

The  microscopical  appearances  of  the  cord  at  the  seat  of 


DISEASES  OF  THE  SPINAL  CORD  357 

the  compression  are  slight— the  axis  cylinders  are  swollen, 
and  the  medullated  sheaths  are  dilated  and  stain  badly.  In 
some  cases  there  are  definite  degenerative  changes,  both  of 
the  nerve  fibres  and  cells.  The  white  matter  is  more  affected 
than  the  grey. 

In  old-standing  cases  secondary  ascending  and  descending 
degeneration  of  the  long  fibre  tracts  is  observed  ;  but  in  many 
cases  the  secondary  degeneration  is  slight  and  the  axis 
cylinders  are  strikingly  preserved. 

Symptoms.  The  disease  being  primarily  one  of  the  bones 
of  the  spine,  affecting  secondarily  the  nerve  roots  and  the 
spinal  cord,  the  symptoms  may  be  conveniently  described 
according  as  they  indicate  lesion  of  the  bone,  the  nerve  roots, 
or  the  spinal  cord. 

1.  Bone  symptoms.  The  earliest  signs  of  spinal  caries  are 
rigidity  of  the  muscles  on  either  side  of  the  affected  vertebrae 
and  a  lack  of  mobility  in  the  vertebral  articulations.  In 
consequence  of  this  a  characteristic  attitude  is  induced, 
which  is  seen  in  the  care  with  which  the  child  bends  the  back, 
as  in  stooping,  or  in  other  actions  requiring  movement  of  the 
spinal  column.  Pain,  either  spontaneous  or  on  movement  of 
the  back,  and  referred  to  a  particular  region  of  the  spine,  is 
a  later  symptom.  It  is  frequently  present  before  any  sign 
of  vertebral  prominence  makes  its  appearance. 

Percussion  of  the  spine  evokes  tenderness  over  one  or  two 
vertebrae,  or  pressing  the  head  or  shoulders  forcibly  down- 
wards will  call  forth  expressions  of  pain  in  the  affected  area. 

Later  on,  a  local  prominence  or  irregularity  of  the  spinous 
processes,  or  a  definite  antero-lateral  curvature,  will  indicate 
the  nature  of  the  disease  and  its  position. 

2.  Boot  symptoms.  Pains  are  rare  in  caries,  but  are  in 
some  cases  an  early  symptom,  and  may  be  present  before  the 
symptoms  of  the  bone  disease  are  apparent.  When  present 
they  are  described  either  as  dull  and  constant  or  as  sharp  and 
radiating  pains  referred  to  the  root  areas  affected  by  the 
disease.  In  cervical  caries  headache  and  pain  referred  to  the 
arms  are  often  associated  with  sympathetic  pupillary  changes. 
In  dorsal  caries  pain  is  referred  to  the  sternum  or  round 
the  body.  In  dorso-lumbar  caries  the  pain  is  abdominal 
or  of  a  girdle  character.  In  lumbar  and  lumbo-sacral  caries 
the  pain  is  referred  to  the  legs  and  genitalia. 


358 


NERVOUS  DISEASES 


3.  Cord  symptom*.  Interference  with  the  functions  of 
the  spinal  cord  is  usually  subsequent  to  the  pain  in  the 
back,  already  described,  but  has  been  known  to  be  the  earliest 
indication  of  the  disease,  especially  in  the  so-called  traumatic 
cases,  where,  owing  to  an  injury  to  the  spine,  paraplegic 
symptoms  have  suddenly  developed.  The  onset  of  paraplegia 
is,  on  the  other  hand,  usually  insidious  ;  and  owing  to  the 


Fig.  112. — Photograph  of  a  case  of  caries  of  the  spine,  with  compression  of 
the  cord  at  the  tenth  dorsal  segment,  showing  drawing  upon  the 
umbilicus  by  the  non-paralysed  portion  of  the  recti  abdominales. 


pressure  being  primarily  upon  the  anterior  or  antero-lateral 
aspects  of  the  cord,  motor  paralysis  alone,  or  motor  weakness 
in  excess  of  sensory  impairment,  is  the  first  sign  of  pressure. 
If  the  abscess  j)i*esses  upon  one  side  of  the  cord,  the  earliest 
symptoms  are  slight  spastic  paresis  on  the  homolateral  side, 
with  slight,  but  definite,  sensory  impairment  on  the  contra- 
lateral side.  Later  on,  complete  motor  and  sensory  paralysis 
will  be  found  below  the  affected  segment  in  severe  cases. 

The  paraplegia  is  of  the  spastic  type,  with  exaggeration 
of  the  deep  reflexes,  abolition  of  the  abdominal  skin  reflexes, 
and  extensor  plantar  responses. 


DISEASES  OF  THE  SPINAL  CORD  359 

Loss  of  sphincter  control  may  not  ensue  until  a  late  stage, 
unless  the  caries  be  sacral  in  position,  when  incontinence 
forms  one  of  the  earliest  of  the  pressure  effects. 

As  already  explained,  when  describing  the  localisation  of 
function  within  the  spinal  cord,  in  addition  to  the  motor  and 
sensory  symptoms  below  the  level  of  compression,  symptoms 
referred  to  the  affected  segment  or  segments  may  also  be 
observed.  These  differ  according  to  the  portion  of  the  cord 
affected.  It  will  therefore  be  convenient  to  refer  to  them 
briefly  in  this  place,  mentioning  at  the  same  time  certain  other 
local  symptoms  of  importance  and  diagnostic  value. 

Symptoms  of  vertebral  caries  in  special  localities 

Occipito-atlo-axoid  caries.  The  head  and  neck  are  held 
stiff,  and  pain  is  complained  of  locally,  or  shoots  over  the 
occiput  on  to  the  vertex.  The  antero-posterior  and  the  lateral 
rotation  movements  of  the  head  are  impaired.  Difficulty  in 
swallowing  may  be  present  from  the  existence  of  a  retro- 
pharyngeal abscess,  or  from  prominence  of  the  vertebral 
bodies. 

Atrophic  paralysis  of  the  muscles  supplied  by  the  spinal 
accessory  nerve,  and  sometimes  of  the  tongue,  have  been 
described. 

Caries  of  the  upper  cervical  region.  In  addition  to  the 
motor  adn  sensory  palsy  below  the  level  of  the  lesion,  palsy  of 
the  diaphragm  from  involvement  of  the  phrenic  nerve  may  be 
observed.  Pain  in  the  neck  and  impairment  in  its  movements 
are  also  present. 

Cervico-dorsal  caries  is  characterised  by  paralysis  and 
wasting  of  certain  of  the  arm,  shoulder,  and  hand  muscles, 
in  addition  to  spastic  paraplegia.  The  atrophic  paralysis 
and  sensory  loss  in  the  arms  will  depend  upon  the  segments 
affected  as  given  in  the  table  upon  p.  318  and  in  the  figures 
90  and  91. 

In  lumbar  caries  the  fibres  forming  the  cauda  equina  are  in 
part  affected,  and  the  symptoms  are  those  of  lesion  of  that 
structure — atrophic  palsy  and  segmental  loss  of  sensation 
corresponding  to  the  affected  nerve  roots  (p.  318).  An 
abscess  may  point  locally  in  the  back,  or  pus  may  pass  along 
the  ilio-psoas  sheath  and  point  below  Poupart's  ligament. 


360  NERVOUS  DISEASES 

Abscess  formation.  In  all  cases  of  vertebral  caries,  the 
possible  presence  of  pus  should  be  borne  in  mind.  The 
abscess  may  either  point  locally,  or  by  passing  along  certain 
lines  of  least  resistance  appear  at  a  considerable  distance  from 
the  disease.  A  retro-pharyngeal  abscess  forms  in  connexion 
with  caries  of  the  upper  cervical  region ;  pus  may  invade  the 
posterior  mediastinum  from  caries  of  the  lower  cervical  or 
upper  dorsal  regions,  and  a  psoas  abscess  may  point  in  the 
groin  from  lower  dorsal  or  lumbar  caries.  Abscess  is  most 
common  in  association  with  lumbar  caries. 

Symptoms  of  tubercle  elsewhere  in  the  body  should 
always  be  sought  for,  both  in  the  early  stages  and  during 
the  prolonged  treatment  which  these  cases  require.  The 
temperature  may  or  may  not  show  an  evening  rise.  Where 
the  disease  is  active,  general  malaise,  cough,  and  an  evening 
rise  of  temperature  may  be  present. 

Diagnosis.  The  diagnosis  of  spinal  caries  presents  no 
difficulty  in  the  majority  of  cases.  The  stiffness  and  rigidity 
of  the  back,  local  tenderness  on  pressure,  root  pains,  and 
symptoms  of  compression  paraplegia  with  irregularity  of  the 
spinous  processes  and  angular  deformity  are  usually  sufficient 
on  which  to  base  the  diagnosis,  especially  when  taken  along 
with  the  general  symptoms. 

Examination  by  the  Eontgen  rays  should  be  carried  out 
in  all  cases  where  possible. 

The  malady  has  to  be  distinguished  from  - 

1.  Hysteria.  In  this  condition  the  absence  of  angular 
deformity  or  of  signs  of  organic  disease,  a  more  diffuse  and 
extensive  spinal  tenderness  elicited  by  light  pressure,  or  not 
obtained  by  deep  pressure  when  the  patient's  attention  is 
distracted,  and  the  presence  of  spinal  mobility  on  pressure 
over  a  tender  area  are  usually  sufficient  to  distinguish  the 
malady. 

2.  Malignant  disease  of  the  vertebral.  The  special 
features  of  this  condition  are  the  age  of  the  patient,  the 
probable  history  of  an  earlier  operation  for  tumour,  the 
presence  of  malignant  disease  elsewhere,  the  diffuse  character 
of  the  pain,  often  worse  when  tying  recumbent,  the  presence 
of  lumps  on  the  bones,  and  the  general  cachectic  condition. 

Prognosis.  The  course  of  the  disease  is  prolonged.  In 
some  cases  vertebral  disease  may  commence  in  childhood  and 


DISEASES  OF  THE  SPINAL  CORD  361 

give  rise  to  curvature,  while  paraplegic  symptoms  only  ensue 
in  adult  life. 

In  other  cases  the  bone  disease  and  the  secondary  spinal 
symptoms  develop  within  a  short  period  of  each  other,  or  may 
even  come  on  simultaneously. 

In  the  great  majority  of  cases,  after  the  development  of 
compression  paraplegia,  the  further  course  of  the  malady  is 
prolonged.  In  some,  recovery  results.  In  others,  permanent 
paralysis  results  with  the  formation  of  bed-sores  and  cystitis ; 
or  general  tuberculosis  and  pyaemia  cause  death. 

Rarer  complications  of  the  disease  are  occasionally  found 
in  hemorrhage  within  the  spinal  cord  and  the  formation  of 
a  central  abscess  (p.  326). 

The  prognosis  is  much  more  favourable  in  children  than 
in  adults. 

Treatment.  In  all  cases  the  general  treatment  of 
tuberculosis,  in  addition  to  the  special  treatment  of  the  local 
condition,  should  be  prescribed.  This  consists  primarily  in 
securing  good  hygienic  conditions  such  as  prevail  in  open-air 
sanatoria,  with  ample  nourishment,  tonics,  and  cod-liver  oil. 

Best  to  the  spine.  Rest  in  the  recumbent  posture  is 
essential  in  most  cases.  This  may  be  rendered  more  satis- 
factory by  the  addition  of  extension  or  mechanical  supports 
to  the  spine.  In  cervical  caries  the  neck  may  be  fixed  by 
means  of  a  collar,  or  by  the  use  of  sand-bags  placed  on  each 
side  of  the  head.  In  dorsal  caries  double  extension  may  be 
adopted,  the  patient,  however,  being  permitted  the  use  of  his 
arms.  Rest  should  be  persevered  with  for  twelve  months, 
or  longer  if  necessary.  In  children  this  will  usually  suffice 
to  relieve  the  cord  of  pressure,  and  procure  the  healing  of 
the  tuberculous  lesion.  In  adults  the  results  are  not  so 
favourable  ;  but  the  method  should  be  given  a  fair  trial. 

Every  care  should  be  taken  to  prevent  the  development 
of  bed-sores,  and  attention  should  be  given  to  the  bladder. 
In  cases  with  reflex  spasms  of  the  legs,  the  bromides,  cannabis 
indica,  phenacetin,  or  veronal  may  be  prescribed. 

Abscesses  should  be  opened  and  washed  out  on  the  lines 
mentioned  in  the  surgical  text-books. 

Should  recovery  occur,  or  when  the  recumbent  posture  is 
no  longer  necessary,  a  support  may  be  supplied  to  strengthen 
the  spine  and  to  remove  the  weight  of  the   body  from  the 


362  NERVOUS  DISEASES 

affected  part.  In  cervical  caries  a  collar  may  be  fitted  ;  and 
in  lower,  dorsal  or  lumbar  caries,  a  spinal  jacket  fitted  with  arm 
supports  resting  upon  the  pelvis  may  be  prescribed.  By  these 
means  both  the  local  and  general  condition  of  the  patient  will 
be  improved. 

The  question  of  laminectomy  in  these  cases  is  one  of  the 
most  difficult  upon  which  to  give  an  opinion.    It  is  indicated — 

(a)  In  cases  where  the  focus  of  the  disease  is  pressing 
upon  the  upper  cervical  region  and  threatening  life. 

(b)  In  cases  where,  with  a  definite  focus,  the  spasticity  of 
the  limbs  is  passing  into  that  of  flaccidity. 

(c)  In  cases  where  there  is  persistent  pain,  associated  with 
sensory  disturbance,  as  in  sacral  caries. 

(d)  In  those  cases  in  which  there  is  a  single  focus  of 
disease,  in  which  symptoms  do  not  resolve  with  rest,  and  in 
which  spastic  contractures  are  steadily  increasing. 

Laminectomy  is  contra-indicated  when  there  are  signs  of 
general  tuberculosis,  or  of  two  or  more  foci  of  pressure  upon 
the  cord.  In  both  these  tj^pes  of  case  operative  interference, 
if  not  immediately  fatal,  may  result  in  the  lighting  up  of 
tuberculous  meningitis.  In  cases  complicated  by  lumbar  or 
psoas  abscess,  this  should  be  opened  and  drained. 

In  successful  cases  a  more  or  less  rapid  recovery  of  the 
pressure  symptoms  after  operation  takes  place.  This,  however, 
should  not  interfere  with  the  carrying  out  of  prolonged  rest 
and  open-air  treatment.  If  the  patient  remains  free  from 
symptoms  for  two  years,  he  may  be  permitted  to  move  about 
a  little,  a  suitable  apparatus  being  supplied  for  the  support 
and  fixation  of  the  spine.  Special  care  should  be  taken  to 
avoid  jars,  concussion,  or  strain  upon  the  back.  The  condition 
of  the  limbs  should  be  attended  to  by  means  of  massage  and 
passive  movements. 

3.  MALIGNANT*  DISEASE  OF  THE  VERTEBRA 

Both  benign  and  malignant  tumours  may  affect  the 
vertebral  column,  but  the  latter  are  the  more  frequent  and 
important.  Carcinoma  and  sarcoma  are  the  commonest  of 
the  malignant  tumours.  Carcinoma  is  invariably  secondary 
to  cancer  elsewhere,  chiefly  of  the  breast ;  sarcoma  is  usually 
primary,  either  of  the  vertebral  bodies,  their  periosteum,  or 


DISEASES  OF  THE  SPINAL  CORD  363 

the  adjacent  soft  parts.  These  tumours  invade  the  structures 
adjacent  to  their  site  of  origin,  in  many  cases  growing  into  the 
spinal  canal,  and  eventually  pressing  upon  the  nerve  roots  in 
the  intervertebral  foramina,  the  dura  mater,  and  the  spinal 
cord.  The  dorsal  region  of  the  spinal  column  is  that  chiefly 
involved  in  malignant  disease. 

Symptoms.  These  may  be  described  according  as  they  are 
referred  to  the  bones  of  the  spine,  the  nerve  roots,  and  the 
spinal  cord. 

(a)  The  bones.  Malignant  disease  of  the  bones  is  cha- 
racterised by  local  pain  and  tenderness  on  pressure,  by 
interference  with  the  movements  of  the  spine,  arising  partly 
from  stiffness,  but  partly  due  to  the  fear  of  causing  pain  by 
movement ;  and  by  deformity,  or  kyphosis,  of  that  portion 
of  the  spine  in  which  the  growth  is  situated. 

The  local  pain  is  sometimes  arrested  in  the  recumbent 
posture,  but  readily  comes  on  in  the  erect  position  and  on 
movement.  An  eminently  characteristic  feature  of  the  pain 
of  malignant  disease  of  the  spine  is  a  tendency  to  remission, 
sometimes  for  long  periods,  under  conditions  of  rest. 

(b)  Nerve  roots.  These  structures  are  affected  either  by 
pressure  or  from  direct  implication  in  the  new  growth.  Root 
pains  are  of  an  intensely  excruciating  character,  and  radiate 
along  the  distribution  of  the  affected  root  or  roots.  Although 
induced  by  movement,  they  are  often  entirely  spontaneous 
in  origin.  They  are  usually  unilateral,  and  may  be  asso- 
ciated with  a  definite  zone  of  hyperesthesia  over  the  area 
of  distribution  of  the  affected  nerve.  If  the  nerve  root 
is  destroyed,  a  corresponding  zone  of  anesthesia  may  be 
detected. 

In  a  number  of  cases  of  malignant  spinal  disease,  the 
symptoms  throughout  the  whole  course  of  the  malady  are 
referred  solely  to  the  bodies  and  the  nerve  roots.  In  others, 
however,  symptoms  of  pressure  upon  the  spinal  cord  develop 
at  an  early  stage. 

(c)  Spinal  cord.  The  onset  of  cord  symptoms  is  usually 
rapid.  They  consist  of  motor,  sensory,  and  reflex  phenomena 
below  the  level  of  compression. 

The  course  and  duration  of  these  cases  is  variable.  The 
interval  which  may  elapse  between  the  primary  disease 
elsewhere  and    the    appearance    of    symptoms   of   secondary 


364  NERVOUS  DISEASES 

metastatic  growths  in  the  spinal  column  is  uncertain,  being 
in  one  case  as  long  as  seven  years.  Once  the  spine  becomes 
affected  the  symptoms  progress  rapidly,  but  life  may  be 
prolonged  for  twelve  or  eighteen  months.  "With  the  onset 
of  paraplegic  symptoms,  and  the  development  of  cystitis  and 
bed-sores,  the  duration  of  life  is  short,  on  an  average  of  about 
two  months. 

The  only  treatment  is  palliative,  as  surgical  interference 
is  out  of  the  question.  Hypodermic  injections  of  morphia  are 
the  best  means  at  our  disposal,  but  aspirin  and  phenacetin 
have  proved  efficacious  in  some  cases.  Complete  rest  in  bed 
is  essential. 

4.  THAUMATIC  LESIONS  OF  THE   SPINAL  COLUMN 
INV0LA7ING  THE  SPINAL  COED 

Traumatic  lesions  of  the  spine  are  of  several  kinds.  The 
following  are  the  most  important :  — 

1.  Extradural  hemorrhage.  This  is  a  complication  of  rare 
occurrence,  resulting  from  severe  strain  or  stress  applied 
to  the  spine.  The  symptoms  depend  upon  the  amount  of 
hemorrhage  and  the  degree  of  pressure  which  the  extra- 
vasated  blood  may  exert  upon  the  spinal  cord  or  the  emerging 
roots.  There  is  a  tendency  for  the  blood  to  gravitate  towards 
the  lower  end  of  the  neural  canal,  in  consequence  of  which 
the  symptoms,  of  which  pain  is  the  chief,  are  mainly  referred 
to  the  lumbo-sacral  roots. 

2.  Fracture  of  the  spine  This  may  be  limited  to  the 
spinous  processes,  the  laminaB,  or  the  transverse  processes, 
and  does  not  necessarily  give  rise  to  any  pressure  symptoms 
upon  the  spinal  cord. 

3.  Dislocation  of  the  spine.  This  results  from  subluxa- 
tion of  a  vertebral  disc,  or  of  a  vertebral  body  nipping  the 
cord,  and  then  recoiling  into  position. 

4.  Fracture-dislocation  is  a  condition  characterised  by 
the  permanent  displacement  of  a  vertebral  body.  The 
symptoms  may  arise  from  hemorrhage  into  the  substance 
of  the  cord,  from  bone  being  driven  into  the  cord,  from 
crushing  of  the  cord,  or  from  a  combination  of  these 
conditions. 

A  solution  of  the  physiological  continuity  of  the  cord  may 


DISEASES  OF  THE  SPINAL  CORD  365 

occur  in  cases  in  which  instantaneous  momentary  nipping  of 
the  cord  has  occurred. 

Incomplete  lesions  of  the  cord  may  result  from  intra- 
medullary hemorrhage  or  bruising,  or  from  extramedullary 
hemorrhage  giving  rise  to  compression,  which  occurs  not 
immediately  at  the  time  of  injury,  but  later  during  the  period 
of  recovery  from  shock. 

The  cord  also  may  suffer  from  ischeemia,  the  result  of 
being  stretched  over,  or  pressed  upon,  a  displaced  vertebral 
body  or  fragment  of  bone. 

Symptoms,  (a)  Complete  anatomical  severance  of  the 
cord.  This  injury  is  followed  by  severe  shock  and  collapse,  and 
is  often  attended  by  unconsciousness.  The  temperature  may 
rise  to  hyperpyrexia.  Complete  motor  and  sensory  paralysis 
is  found  below  the  level  of  the  lesion,  with  abolition  of  all  the 
reflexes  and  retention  of  urine.  At  the  upper  limit  of  the 
lesion  a  zone  of  hyperesthesia  may  be  found.  In  a  certain 
number  of  cases  sugar  is  found  in  the  urine.  Trophic  changes 
are  especially  liable  to  occur. 

Death  may  occur  during  the  period  of  collapse,  or  may 
follow  in  from  one  to  three  weeks  from  septic  absorption  from 
bed-sores  or  cystitis. 

(b)  Incomplete  anatomical,  but  complete  physiological 
severance  of  the  cord.  The  immediate  effects  are  similar  to 
those  seen  in  the  complete  cases.  Should  the  patient  recover 
from  the  effects  of  the  injury,  improvement  is  first  seen  in 
a  return  of  the  reflexes,  the  plantar  returning  early  and  of 
the  extensor  type,  and  followed  by  the  restoration  of  the  deep 
reflexes.  After  a  few  days,  retention  of  urine  gives  place 
to  reflex  incontinence. 

In  upper  dorsal  and  cervical  lesions  priapism  may  be  a 
troublesome  complication,  and  in  cervical  cases  optic  neuritis 
has  been  observed  as  a  rare  phenomenon. 

The  after- stages  are  characterised  by  the  resolution  of  the 
trophic  lesions,  the  healing  of  bed-sores,  the  onset  of  spasticity 
and  reflex  spasms  in  the  paralysed  limbs,  which  may  have 
shown  rapid  wasting.  The  sensory  symptoms  also  become 
less  marked — the  hyperesthesia  lessens  and  the  sensory  loss 
diminishes.  Voluntary  power  returns  slowly,  commencing  in 
the  distal  portions  of  the  limbs. 

(c)  Incomplete    lesions    with    secondary   hemorrhage.     In 


366  NERVOUS  DISEASES 

slighter  cases,  a  partial  motor  and  sensory  paralysis  below 
the  level  of  the  lesion  niay  be  detected.  In  these  cases  it  is 
important  to  observe  the  subsequent  state  of  the  patient, 
as  in  some  instances  more  serious  damage  takes  place  later 
from  the  occurrence  of  hemorrhage  with  secondary  pressure 
symptoms.  The  importance  of  this  bears  upon  treatment, 
as  in  any  case  in  which  the  reflexes,  present  after  the 
accident,  subsequently  become  lessened  or  abolished,  and  the 
motor  and  sensory  symptoms  increase,  surgical  interference 
should  be  at  once  adopted,  as  the  recent  symptoms  are 
probably  due  to  hemorrhage. 

Symptoms  pointing  to  an  extramedullary  hemorrhage  are: 
an  increase  in  the  zone  of  hyperesthesia,  intensification  of 
the  local  segmental  symptoms,  augmentation  of  the  motor 
paralysis  and  sensory  loss  below  the  lesion,  and  a  disappear- 
ance of  the  deep  reflexes,  which  may  have  been  increased 
during  the  initial  stage  subsequent  to  the  collapse. 

The  symptoms  of  an  increasing  intramedullary  lesion  are 
the  development  of  marked  paralysis  of  the  Brown-Sequard 
type,  of  dissociation  of  sensation,  and  of  gradually  increasing 
paraplegia. 

(d)  Sij2^mtoms  referred  to  a  local  ischcemic  condition  of 
the  cord.  The  early  symptoms,  which  may  have  been  slight, 
resolve  to  a  greater  or  less  extent ;  but  after  a  few  weeks, 
progress  is  arrested  and  an  increase  of  the  paraplegic 
symptoms  takes  place,  especially  motor  weakness  associated 
with  increasing  spasticitj7  and  reflex  spasm.  The  important 
feature  of  this  condition  is  the  increase  of  spasticity 
and  spasm,  accompanied  by  increasing  motor  weakness — an 
indication  of  retrogressive  action.  It  ought  to  be  distin- 
guished from  an  increase  of  spasticity  associated  with  the 
increase  of  voluntary  power  —  a  sign  of  resolution  and 
progress.  Increasing  weakness,  therefore,  indicates  pressure 
upon  the  spinal  cord,  either  from  the  effects  of  organising 
and  contracting  blood  -  clot,  or  from  the  long  -  sustained 
stretching  of   the  cord  over  a  bony  prominence. 

The  condition  closely  resembles  that  seen  in  certain  cases 
of  Pott's  disease  of  the  vertebra?,  in  dry  caries,  in  some 
meningeal  lesions,  and  in  cases  of  slow  compression  from 
any  other  cause. 

The  condition  of  the  spinal  column  in  cases  of  fracture- 


DISEASES  OF  THE  SPINAL  CORD  367 

dislocation  varies.  Fracture  of  the  spines  and  laminae  can 
usually  be  detected  by  palpation,  but  in  all  cases  it  is  advisable 
to  make  use  of  the  X-rays,  which  often  afford  direct  indications 
for  the  immediate  treatment.  It  is  important  to  bear  in 
mind  that  there  is  no  constant  relation  between  the  severity 
of  the  vertebral  lesion  and  the  damage  to  the  spinal  cord. 

Prognosis.  The  complete  cases  end  fatally,  within  six 
weeks  to  three  months,  except  in  those  high  up  in  the  cervical 
region,  when  death  may  be  instantaneous  or  occur  in  a  few 
hours. 

In  the  incomplete  cases,  recovery  depends  upon  the 
damage  to  the  cord  and  the  early  relief  of  pressure  symptoms. 
On  the  one  hand,  recovery  may  be  complete,  or  may  be  to  the 
extent  of  a  spastic  paralysis,  the  patient  being  able  to  walk 
even  without  sticks.  On  the  other  hand,  a  large  number  of 
cases  improve  sufficiently  to  be  able  to  get  about  with 
the  aid  of  sticks  or  crutches.  The  remainder  become 
bedridden. 

The  ultimate  result  depends  especially  upon  the  nursing 
and  management,  the  prevention  of  septic  infection  from 
bed-sores  and  cystitis,  the  maintenance  of  nutrition,  the  pre- 
vention of  contractures,  and  the  conservation  and  fostering  of 
voluntary  motor  power.  It  is  important  to  remember  that 
recovery  may  not  take  place  until  some  years  have  elapsed 
after  the  injury. 

Treatment.  This  depends  in  large  measure  upon  the 
diagnosis  and  the  appreciation  of  the  nature  and  situation  of 
the  local  injury.  During  the  stage  of  collapse  nothing  ought 
to  be  done,  unless  the  situation  of  the  lesion  is  immediately 
dangerous  to  life,  as  in  high  cervical  injuries,  where  paralysis 
of  the  phrenic  nerve  calls  for  urgent  relief.  After  the  stage 
of  collapse  in  cases  of  severe  injury,  appropriate  treatment 
such  as  removal  of  fragments  of  bone  should  be  adopted. 
In  cases  of  less  obvious  injury,  where  an  X-ray  examination 
may  reveal  fracture  or  dislocation,  or  in  the  cervical  region 
blood  -  clot  within  the  neural  canal,  operation  should  be 
carried  out  at  once.  If,  on  the  other  hand,  the  nervous 
symptoms  are  resolving  satisfactorily,  interference  may  be 
postponed,  with  a  view  to  an  improvement  in  the  patient's 
condition.  In  all  cases  where  an  increase  in  the  nervous 
symptoms   occurs,  or  severe   local   root  pains   are   present, 


368  NERVOUS  DISEASES 

operation  should  be  performed  at  once,  as  delay  may  rob  the 
patient  of  any  chance  of  complete  recovery. 

There  remains  the  not  inconsiderable  number  of  cases  in 
which  no  definite  local  evidence  of  fracture  or  dislocation  is 
present,  and  in  which  the  paraplegic  symptoms  are  character- 
istic more  of  an  intra-  than  an  extramedullary  lesion.  In 
these  cases  it  is  wise  to  watch  the  progress  of  the  case. 
In  intramedullary  lesions  the  symptoms  tend  to  concentrate 
themselves  and  to  become  characteristic  (Brown- Sequard's 
symptom  and  dissociation  of  sensibility).  In  extramedullary 
cases  the  tendency  is  towards  augmentation  or  diffusion  of 
symptoms,  increasing  root  pains,  and  bilateral  motor  paralysis 
and  sensory  loss. 

In  all  cases  the  patient  should  be  placed  on  a  water-bed, 
and  special  care  taken  to  prevent  bedsores,  which  are  prone 
to  occur.  The  catheter  should  be  used,  a  low  diet  prescribed, 
and  contractures  minimised  or  prevented  by  massage,  passive 
movements,  and  extension. 


Paet  IX 

GENERAL   DISEASES   OF   THE   NERVOUS 
SYSTEM 

Chapter  I 
SYPHILITIC   DISEASES 

Syphilis  may  affect  the  nervous  system  in  two  ways  : — 

First,  it  causes  a  specific  inflammatory  affection  of  the 
blood-vessels  and  membranes  of  the  brain  and  spinal  cord. 
These  changes  show  all  the  histological  features  exhibited 
by  syphilis  elsewhere,  and  bring  about  alterations  in  the 
nervous  tissues,  either  by  diminishing  or  arresting  the 
blood  supply  through  vascular  occlusion,  or  by  the  direct 
effect  of  the  pressure  of  new  formations  (gummata)  upon 
the  nervous  elements — syphilitic  lesions. 

Secondly,  it  exerts  a  specific  action  upon  the  vital  energy 
of  various  component  parts  of  the  nervous  system.  Although 
this  action,  as  far  as  our  present  knowledge  goes,  is  not  fully 
understood,  it  would  appear  to  be  the  result  of  a  toxic 
agent  produced  within  the  system.  The  histological  changes 
found  in  these  cases  are  distinct  from  those  of  true  syphilitic 
lesions,  and  consist  of  a  primaiy  degeneration  of  various  cells 
and  fibre  tracts  of  the  nervous  system— parasyphilitic  lesions. 

Symptoms  of  syphilitic  lesions  of  the  nervous  system  may 
be  observed  at  any  period  after  infection.  According  to  Mott 
there  is  no  period  at  which  syphilitic  brain  disease  may  not 
occur,  but  the  most  intractable  to  treatment  and  the  most 
severe  and  generalised  forms  occur  within  two  or  three  years 
after  infection. 

2-i 


370 


NERVOUS  DISEASES 


Table    A    shows   the    time   of    onset   of    symptoms    after 
infection  in  34  cases  of  cerebro- spinal  S3rphilis. 


Years  after 
infection 

1—2 

2-4 

4-5 

6-8 

8-10 

over  10 

No.  of  cases 

7 

4 

3 

3                 3 

14 

In  contrast   to  this,  Table  B  shows  the  time  of  onset  of 
symptoms  after  infection  in  65  cases  of  tabes  dorsalis. 

Years  after 
infection 

1—5 

5-10 

10—15 

15—20 

over  20 

No.  of  cases 

2 

8 

17 

23 

15 

The  general  statement  may  be  made  that  true  syphilitic 
lesions  occur  early,  and  the  degenerative  or  parasyphilitic 
develop  much  later. 


CEREBRO-SPINAL    SYPHILITIC    LESIONS 

Etiology.  In  the  majority  of  cases  no  immediate  cause 
is  present.  In  a  few  cases,  however,  an  injury  may  favour 
the  development  of  a  local  syphilitic  lesion. 

Varieties.  Cerebro-spmal  syphilitic  lesions  are  of  the 
following    kinds : — 

1.  Vascular  lesions. 

2.  Syphilitic  meningitis — (a)  leptomeningitis  ; 

(b)  pachymeningitis. 

3.  Gummata. 

4.  Diffuse  lesions — gummata,  endarteritis,  meningitis. 

I.  Vascular  lesions.  The  vessels  most  commonly  affected 
are  those  of  the  circle  of  Willis  and  the  Sylvian  fossa,  the 
basilar  and  the  lenticulo-striate  arteries.  The  characteristic 
structural  change  consists  of  a  proliferation  of  the  intima, 
with  obliteration  or  narrowing  of  the  lumen.  Similar  changes 
are  also  seen  in  the  middle  and  adventitial  coats.  The  elastic 
lamina  may  become  broken  up  and  separated  by  proliferated 
cells.  In  some  cases  small  gummata  form  in  the  vessel  wall. 
Perivascular  cellular  infiltration  occurs  in  most  cases. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    371 

The  effect  of  these  vascular  changes  depends  upon  their 
degree  and  situation  in  the  affected  blood-vessel.  When  the 
lesion  is  localised,  a  thrombus  may  form,  and  give  rise  to 
softening  in  the  distribution  of  the  affected  artery.  Not 
infrequently,  before  an  actual  occlusion  takes  place,  symp- 
toms pointing  to  a  transient  functional  disturbance  permit  of 
its  recognition  and  the  employment  of  active  antisyphilitic 
treatment. 

The  fact  that  syphilis  is  the  predominant  cause  of  vascular 
disease  in  .early  life  should  always  be  remembered,  and  in 
any  doubtful  case  a  cytological  examination  of  the  cerebro- 
spinal fluid  should  be  made. 

The  symptoms  arising  from  this  lesion  may  be  referred  to 
any  part  of  the  central  nervous  system. 

2.  Syphilitic  meningitis,  (a)  Leptomeningitis.  Syphilitic 
disease  of  the  pia-arachnoid  membrane,  though  frequent,  is 
often  overlooked.  It  may  involve  the  cerebral  or  spinal 
membranes. 

In  the  brain  leptomeningitis  causes  an  opaque  indura- 
tion of  the  membrane,  often  with  small  nodules  resembling 
tuberculous  meningitis,  and  invariably  associated  with  syphilitic 
changes  in  the  superficial  blood-vessels.  It  is  also  found 
associated  with  more  advanced  changes — gummata,  gummatous 
meningitis  or  vascular  lesions,  such  conditions  giving  rise 
to  epilepsy,  or  to  Jacksonian  convulsions. 

The  symptoms  are  headache,  especially  at  night,  and 
irritative  phenomena  corresponding  to  the  locality  of  the 
lesion.  If  over  the  occipital  lobe,  visual  spectra  in  the  opposite 
half-fields  are  described  ;  if  over  the  motor  region,  Jacksonian 
attacks  in  the  opposite  limbs.  Paralytic  symptoms  succeed 
the  irritative,  and  result  from  the  destruction  of  the  nerve 
elements  of  the  underlying  cortex,  by  extension  inwards  of 
the  gummatous  process. 

In  the  spinal  cord  its  presence  as  a  definite  pathological 
factor,  capable  of  giving  rise  to  characteristic  clinical  symptoms, 
has  not  been  fully  appreciated.  Certain  cases  of  slowly 
progressive  paraplegia  with  sensory  disturbances  to  a  definite 
segmental  level,  in  wThich  the  diagnosis  of  compression 
paraplegia  due  to  a  tumour  has  been  made,  have  been  found 
on  operation  to  arise  from  a  constriction  of  the  cord,  with 
damming  up  of  the  cerebro-spinal  fluid  from  a  local  lepto- 

24  * 


372  NERVOUS  DISEASES 

meningitis.  That  such,  and  not  a  secondary  intramedullary 
lesion,  was  the  cause  is  probable  from  the  fact  that  recovery 
has  resulted  from  the  division  and  freeing  of  the  adhesions.1 
In  this  connexion  it  is  right  to  mention  that  the  associ- 
ation of  leptomeningitis  with  syphilitic  endarteritis  and 
spinal  thrombosis  is  well  recognised  as  a  common  cause  of 
myelitis. 

(b)  Pachymeningitis.  This  is  an  inflammatory  affection  of 
the  dura  mater.  It  is  the  commonest  variety  of  syphilitic 
meningitis.     It  may  be  general  or  circumscribed. 

In  the  brain  it  is  most  frequent  over  the  base,  especially 
in  the  interpeduncular  space,  when  it  gives  rise  to  headache, 
drowsiness,  vomiting,  and  various  cranial  nerve  affections  — 
paralysis  of  the  third,  sixth,  and  optic  nerves.  If  on  the 
convexity,  it  may  be  primarily  dural,  or  secondary  to  an 
extension  from  the  bones  of  the  skull.  If  circumscribed,  it  is 
not  infrequently  located  in  the  region  of  an  old  injury. 

In  the  spinal  cord  pachymeningitis  affects  especially 
the  cervical  and  lumbo-sacral  regions  and  the  cauda  equina. 
Clinically,  this  condition  is  characterised  by  severe  root  pains, 
atrophic  motor  paralyses,  and  segmental  anaesthesias,  corre- 
sponding to  the  affected  roots,  and  when  there  is  compression 
of  the  cord,  spastic  paraplegia  and  sensory  impairment  below 
the  level  of  the  lesion. 

3.  Gummata.  A  single  gumma  is  rare,  and  only  arises 
from  the  direct  extension  of  a  circumscribed  gummatous 
pachymeningitis  into  the  brain  substance.  Multiple  gummata 
are  common,  and  invariably  arise  from  the  pia  arachnoid,  or 
the  adventitia  of  a  cortical  blood-vessel.  The  changes  in  the 
cerebral  tissue  in  the  neighbourhood  of  a  gumma  consist  of  a 
perivascular  infiltration,  an  increase  of  the  connective  tissue 
of  the  pial  processes,  degeneration  of  the  nerve  elements, 
and  secondary  proliferation  of  the  neuroglia  with  numerous 
spider  or  Deiters's  cells. 

Symptoms.  The  symptoms  of  single  large  gummata  are 
those  of  intracranial  tumour.  When  multiple,  the  symptoms 
are  diffuse  with  headache  and  profound  mental  change. 
Convulsions,  local  or  general,  are  present  in  the  early  stages, 
and  are  later  replaced  by  motor  and  sensory  paralysis. 

1  Horsley  (Sir  Victor),  Brit.  Med.  Journal,  1907. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    373 

4.  Diffuse  syphilitic  lesions — cerebro-spinal  syphilis.  In 
the  severer  forms  of  cerebral  syphilis  two  or  more  of  the 
above  described  pathological  changes  are  combined.  Basal 
meningitis  is  common,  and  its  symptoms  may  either  precede 
or  follow  evidence  of  vascular  occlusion.  The  occurrence  of 
fits  of  a  general  epileptic  character  may  be  ascribed  to  a 
diffuse  syphilitic  infection,  or  to  an  increase  of  intracranial 
pressure  in  association  with  a  basal  meningitis.  Jacksonian 
attacks,  or  localised  focal  seizures,  arise  from  a  circumscribed 
gummatous  patch. 

In  many  cases  the  symptoms  point  to  a  limitation  of  the 
lesion  to  the  intracranial  contents,  but  not  infrequently 
these  are  combined  with  signs  of  spinal  meningitis.  On  the 
other  hand,  a  small  number  of  cases  commence  with 
spinal  symptoms,  which  advance  to  a  considerable  severity 
prior  to  the  onset  of  symptoms  indicating  intracranial 
disease. 

The  clinical  features  of  the  diffuse  cerebral  type,  a  con- 
dition of  importance  owing  to  its  close  resemblance  to  general 
paralysis,  are  the  following  :  The  early  appearance  of  cranial 
nerve  paralyses,  especially  of  the  third  and  sixth  nerves, 
characteristic  pupillary  changes  (irregularity,  inequality,  and 
impaired  light-reaction),  the  occurrence  of  general  or  localised 
epileptic  seizures,  often  followed  by  paralysis  with  definite 
signs  of  organic  disease.  The  general  symptoms  of  intra- 
cranial tumour  (headache,  vomiting,  and  optic  neuritis)  may 
be  superadded,  and  marked  mental  impairment  is  present 
from  an  early  stage.  This  form  of  cerebral  syphilis  is  dis- 
tinguished from  progressive  general  paralysis  of  the  insane 
by  the  absence  of  the  peculiar  mental  state  of  general  paralysis, 
the  early  evidence  of  gross  intracranial  disease,  as  shown  by 
the  paralysis  of  the  cranial  nerves  and  alterations  in  the 
reflexes,  the  absence  of  tremor  and  of  slurring  speech,  and 
the  favourable  response  to  energetic  antisyphilitic  treatment. 

Further,  there  is  in  diffuse  cerebro-spinal  syphilis  a  lesser 
degree  of  lymphocytosis  in  the  cerebro-spinal  fluid,  and 
the  Wasserman  reaction  is  either  feeble  or  not  obtained 
(p.  406). 

Prognosis.  The  prognosis  of  these  cerebro-spinal  syphi- 
litic affections  is  variable,  much  depending  upon  the  amount 
of    destruction    of    the    nerve    elements    which    has    taken 


.',71  NERVOUS  DISEASES 

place.  The  most  unsatisfactory  type  of  the  malady  is  that 
associated  with  multiple  lesions  (syphilitic  dementia),  especially 
when  it  occurs  within  a  few  years  of  infection.  The  vascular 
lesions  are  not  unfavourable  apart  from  the  permanent 
destructive  effects  upon  the  nerve  tissue.  The  basal  lesions 
affecting  the  cranial  nerves,  especially  if  of  limited  extent, 
are  relatively  favourable  when  submitted  to  early  and 
thorough  treatment.  The  most  favourable  of  the  intra- 
cranial lesions  are  those  arising  from  gummatous  meningitis 
of  the  convexity. 

When  estimating  the  prognosis  of  cerebral  syphilis,  the 
tendency  to  remission  and  relapse  of  the  symptoms  has  to  be 
borne  in  mind. 


SYPHILITIC    MYELITIS    (SOFTENING   OF   THE 
SPINAL    CORD:   MYELOMALACIA) 

Probably  the  most  frequent  cause  of  so-called  acute  myelitis 
is  a  diseased  condition  of  the  spinal  blood-vessels  with  throm- 
bosis and  consequent  softening. 

The  occurrence  of  acute  myelitis  from  other  causes,  such 
as  bacterial  infection,  is  relatively  rare  and  is  elsewhere 
described  (p.  320).  Spinal  thrombosis  therefore,  giving  rise 
to  a  type  of  acute  myelitis,  occurs  either  in  association  with 
syphilitic  vascular  lesions,  or  with  atheroma. 

As  the  clinical  picture  of  spinal  thrombosis  is  more  or 
less  similar  whatever  the  cause,  we  propose  to  describe  the 
syphilitic  variety  as  the  type. 

Syphilitic  myelitis  may  be  a  primary  condition,  or  may  be 
one  of  several  manifestations  of  the  action  of  the  syphilitic 
virus.  This  form  of  myelitis  may  be  associated  wTith  the 
pupillary  changes  characteristic  of  syphilis,  and  even  with 
hemiplegia  or  tabes  dorsalis.  It  is  rarer  than  cerebral 
thrombosis,  and  although  it  may  be  unattended  by  clinical 
evidence  of  spinal  meningitis,  some  degree  of  meningeal 
affection  is  usually  present  on  postmortem  examination. 

Symptoms.  The  onset  of  paralysis  may  be  sudden,  but 
premonitory  symptoms  such  as  tingling  in,  or  transient 
weakness  of,  the  lower  limbs  are  rarely  absent,  although  in 
some  cases  they  are  slight  and  unassociated  in  the  patient's 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    375 

mind  with  the  onset  of  the  subsequent  and  graver  paraplegia. 
Although  a  chill,  wetting,  or  fatigue  may  be  tHe  assigned 
cause,  a  history  of  slight  premonitory  symptoms  is  usually 
obtained. 

The  paraplegia  develops  suddenly,  with  loss  of  motion 
and  of  sensation  below  a  definite  level,  usually  in  the  dorsal 
region  of  the  cord,  and  with  retention  of  urine.  Although  the 
symptoms  are  commonly  bilateral,  paralysis  of  the  Brown - 
Sequard  type  is  occasionally  present. 

If  the  lesion  is  total,  the  paralysis  is  flaccid  with  loss  of 
the  deep  reflexes.  More  commonly  the  reflexes  are  not  lost, 
and  may  even  be  increased  from  the  onset  and  associated  with 
slight  spasticity.  Hyperesthesia  at  the  upper  level  of  the 
lesion  may  be  present. 

In  severe  cases,  with  complete  flaccid  paraplegia,  bed-sores 
and  septic  infection  may  ensue  and  cause  death.  If  this 
danger  is  averted,  recovery  gradually  sets  in,  the  stages  of 
resolution  being,  first  a  return  of  the  deep  reflexes,  then  the 
substitution  of  incontinence  for  retention  of  urine,  and  later 
the  gradual  restoration  of  sensation.  In  from  three  to  five 
months  sensibility  may  be  entirely  restored.  During  this  time 
a  slow  and  progressive  return  of  motor  power  takes  place, 
commencing  with  movements  of  the  toes,  and  later  of  the 
whole  limb.  The  most  troublesome  of  the  motor  symptoms 
is  the  occurrence  of  spasms,  either  extensor  or  flexor,  more 
commonly  the  latter,  along  with  the  development  of  spasticity. 
The  limbs  become  flexed  and  adducted  at  the  hips,  and  the 
feet  pointed,  while  sores  develop  from  the  pressure  of  the 
knees  against  each  other  and  on  the  heels  and  sacrum. 

The  muscles  show  general  wasting  without  electrical 
changes,  and  the  skin  becomes  dry  and  atrophic. 

With  proper  care  and  nursing  contractures  may  bo 
prevented  or  reduced  to  a  minimum,  and  the  patient  enabled 
to  walk  with  or  without  assistance,  the  gait  being  of  the 
spastic  type. 

In  the  less  severe  cases  recovery  may  be  almost  complete, 
the  patient  walking  within  about  three  months  of  the  onset, 
but  exhibiting  in  many  instances  slight  weakness  on  one 
side. 

Cystitis  may  arise  from  catheterisation,  or  from  infec- 
tion from  bed-sores.     This  may  lead  to  suppurative  pyelitis 


376  NERVOUS  DISEASES 

and  death  from  uraniia.     Pulmonary  complications,  such  as 
hypostatic  congestion  and  bronchitis,  are  troublesome. 

The  points  of  value  in  the  differential  diagnosis  of  this 
condition  are :  (a)  Pupillary  changes  (inequality,  irregularity  of 
outline,  sluggish  light-reaction  or  reflex  iridoplegia)  character- 
istic of  syphilis,  (b)  Absence  of  nystagmus,  but  impairment 
of  the  ocular  movements,  (c)  Normal  motion  and  sensation 
above  the  level  of  the  lesion,  except  for  a  zone  of  hyperesthesia. 
(<1)  Below  the  level  of  the  lesion,  motor  paralysis  (flaccid  or 
spastic)  and  loss  or  impairment  of  all  forms  of  sensibility, 
sometimes  having  the  features  of  the  Brown-Sequard 
'symptom-complex.'  (e)  The  deep  reflexes  are  eventually 
exaggerated,  with  clonus,  extensor  plantar  response,  and 
absent  abdominal  reflexes  below  the  level  of  the  lesion.  (/) 
At  first  retention  of  urine  and  involuntary  evacuation  of 
faeces,  followed  later  by  reflex  incontinence  and  obstinate 
constipation. 

HYPERTROPHIC  CERVICAL  PACHYMENINGITIS 

This  is  a  condition  characterised  pathologically  by  chronic 
inflammation  and  thickening  of  the  inner  layer  of  the  dura 
mater  and  of  the  soft  membranes  which  surround  the  spinal 
cord  in  the  cervical  region.  In  our  experience  it  is  a  syphi- 
litic condition,  although  it  has  been  ascribed  by  some  writers 
to  trauma  of  the  spine.  Some  cases  are  complicated  by  cavity 
formation  within  the  cord. 

Symptoms.  The  onset  is  usually  subacute,  the  early 
symptoms  being  due  to  involvement  of  the  nerve  roots, 
sensory  disturbances  preceding  the  motor.  These  are  at  first 
unilateral,  but  later  become  bilateral.  Pain  in  the  neck 
extends  to  the  occipital  and  interscapular  regions,  and  is 
often  associated  with  rigidity  and  pain  on  movement,  and 
sometimes  by  tenderness  on  pressure  over  the  cervical 
vertebrae. 

The  root  pains  may  be  associated  with  hyperesthesia,  and 
later  with  loss  of  sensation,  and  are  referred  to  the  upper  limbs. 
The  pain  may  persist  for  months,  and  is  followed  by  atrophic 
paralysis,  especially  affecting  the  intrinsic  muscles  of  the 
hand  and  the  flexors  of  the  wrist  and  fingers.  The  hand 
therefore  assumes  a  position  of  hyperextension  at  the  wrist, 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM     :577 

extension  of  the  fingers  at  the  metacarpophalangeal,  and 
flexion  at  the  interphalangeal  joints  (preacher's  hand). 

Cervical  sympathetic  symptoms  may  ensue  early,  at  first 
of  an  irritative  character,  with  dilatation  of  the  pupil,  ex- 
ophthalmos, widening  of  the  palpebral  fissure,  and  sweating 
over  the  same  side  of  the  face ;  later  by  paralytic  phenomena 
— contraction  of  the  pupil,  ptosis,  enophthalmos,  and  absence 
of  sweating. 

As  the  pressure  upon  the  cord  increases,  symptoms  of 
spastic  paraplegia  develop  with  sphincter  trouble,  and  in 
extreme  cases  objective  sensory  loss. 

Prognosis.  Many  cases  tend  to  progress  and  end  fatally, 
but  a  certain  number  become  arrested  with  diminution  of  the 
root  pains,  and  some  recovery  of  power. 

The  treatment  consists  mainly  of  rest,  local  inunction  of 
mercury  and  large  doses  of  the  iodides  internally,  combined 
with  radiant  heat  or  sulphur  baths.  If  the  disease  progresses 
despite  treatment,  surgical  interference  may  be  attempted  and 
an  endeavour  made  to  loosen  and  free  the  affected  membranes. 
A  continuance  of  the  mercurial  treatment  is  advisable,  even 
after  operation.  Where  ordinary  remedies  fail  to  relieve  the 
pain,  division  of  the  posterior  nerve  roots  is  recommended. 
Atrophic  and  paralytic  symptoms  should  be  combated  with 
massage,  passive  movements,  and  appropriate  electrical 
treatment. 

GUMMA  OF  THE  CORD 

Localised  gumma  of  the  spinal  cord  is  rare,  but  gummatous 
meningitis  with  extension  into  the  cord  along  the  blood- 
vessels or  pial  processes  is  common. 

SYPHILITIC  DISEASE  OF  THE  BONES 

Syphilitic  disease  of  the  bones  of  the  spine  is  rare.  It 
occurs  in  the  form  of  exostoses  or  periosteal  nodes,  but 
rarely  gives  rise  to  caries. 

Syphilitic  disease  of  the  bones  of  the  skull  may  give 
rise  to  a  diffuse  dense  sclerosis,  to  localised  thickenings  and 
exostoses,  to  necrosis  or  to  local  thinning  (cranio-tabes). 
Syphilitic  periostitis  of  the  other  bones,  especially  of  the 
forearm,  may  involve  the  nerves  secondarily  b}T  pressure. 


378  NERVOUS  DISEASES 


Tkkat.mkxt  ov  Ckkkhkd-Si'ixai,  Syphilitic   Lesions 

Syphilitic  affections  of  the  nervous  system,  are  treated  on 
the  same  lines  as  similar  lesions  elsewhere  in  the  body.  The 
success  of  treatment  depends  to  a  large  extent  upon  the 
carrying  out  of  certain  general  principles.  First,  treatment 
should  be  commenced  as  soon  as  the  nature  of  the  disease 
is  recognised ;  secondly,  it  should  be  of  an  intermittent 
character,  the  specific  remedies  being  given  for  periods  of 
about  two  months,  and  followed  by  tonic  treatment;  thirdly, 
treatment  should  be  continued  for  a  period  of  over  two  years. 

The  following  are  the  drugs  and  their  methods  of  adminis- 
tration :  — 

I.  Mercury.  This  may  be  given  either  by  the  mouth, 
by  inunction,  or  b}r  intramuscular  injection. 

(a)  By  the  mouth.  If  prescribed  in  this  way  the  best 
preparations  of  mercury  are  corrosive  sublimate  (gr.  ,'.,-  to  -}.) 
and  the  proto-iodide  (green  iodide  of  mercury)  (gr.  ^  to  1).  The 
disadvantages  of  the  ingestion  method  are  uncertainty  in  the 
action  of  the  drugs,  irregularity  in  their  administration,  and 
a  tendency  towards  gastro-intestinal  troubles. 

(b)  Inunction.  This  is  the  method  used  at  Aachen  and 
other  spas.  One  to  one-and-a-half  drachms  of  blue  ointment, 
or  the  ung.  hydrarg.  oleate,  may  be  rubbed  into  the  skin, 
preferably  after  a  hot  bath.  The  rubbing  should  take  about 
fifteen  or  twenty  minutes,  and  different  portions  of  the  body 
should  be  used  on  consecutive  days.  A  course  usually  con- 
sists of  sixty  daily  rubbings. 

(c,  Intramuscular  injections.  This  method  is  strongly  advo- 
cated by  some  authorities.  Its  advantages  are  that  a  known 
quantity  of  the  drug  is  administered  and  absorbed,  that 
smaller  doses  may  be  given,  and  that  less  frequent  appli- 
cations are  necessary.  Injections  of  the  soluble  salts  require 
to  be  given  more  often  than  the  insoluble,  and  their  appli- 
cation is  more  painful.  The  soluble  preparations  are  : 
hydrarg.  perchlor.,  gr.  | ;  hydrarg.  succinimide,  gr.  7\  to  \  ; 
and  hydrarg.  cyanid.,  gr.  \  to  -K 

The  insoluble  preparations  are :  metallic  mercury  in  doses 
of    1   grain,    prescribed   according    to   Lambkin's l    method ; 

1  Lambkin,  System  of  Syphilis,  1908. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    379 

salicylate  of  mercury,  gr.  I ;  and  calomel,  gr.  \  tof .  These  may 
be  prescribed  about  once  weekly  over  a  period  of  two  months. 
Mercury  should  be  persevered  with  until  salivation  occurs, 
provided  that  the  general  state  of  the  patient  is  maintained. 

2.  The  iodides.  These  may  be  prescribed,  either  as  the 
potassium  or  the  sodium  salt,  in  doses  increasing  from  5  to 
20  or  30  grains  thrice  daily.  When  they  are  given  by  the 
mouth  in  conjunction  with  inunction  of  mercury,  highly 
satisfactory  results  are  obtained  in  the  treatment  of  cerebro- 
spinal syphilitic  lesions. 

Iodides  are  of  especial  value  in  the  treatment  of  the  later 
manifestations  of  the  disease. 

3.  Arsenical  preparations.  A  number  of  new  remedies, 
having  arsenic  as  their  chief  ingredient,  have  been  lately 
introduced,  mainly  in  consequence  of  the  researches  of  Neisser, 
Hoffmann  and  others,  upon  the  treatment  of  experimentally 
induced  syphilis.  Atoxyl,  arsacetin,  and  soamin  may  be 
given  either  by  the  mouth  or  by  intramuscular  injection. 
Serious  toxic  effects  (e.g.  blindness)  have  been  recorded  in 
consequence  of  their  use.  It  is,  however,  too  soon  to  express 
any  opinion  upon  their  action  in  the  cure  of  syphilis.  It  is 
possible  that  they  may  serve  as  efficient  tonic  remedies  after 
a  course  of  mercurial  treatment. 

Local  treatment.  In  conjunction  with  the  general  thera- 
peutic measures  just  described,  treatment  of  the  paralysed 
limbs  by  aid  of  massage,  passive  movements,  and  the  faradic 
current  should  be  carried  out  over  long  periods  of  time. 


Chapter  II 
PARASYPHILITIC  DISEASES 

1.  TABES  DORSALIS  (LOCOMOTOR  ATAXY) 

Tabes  dorsalis  is  one  of  the  commonest  chronic  diseases 
of  the  nervous  system.  It  is  essentially  an  affection  of  the 
sensory  neurones,  but  as  such  involves  the  functions  of 
the  nervous  apparatus  as  a  whole. 


380  NERVOUS  DISEASES 

The  characteristic  morbid  changes  are  degeneration  of 
the  posterior  nerve  roots,  of  the  fibres  which  pass  from 
them  into  the  posterior  columns  of  the  spinal  cord,  and  of  the 
sensory  fibres  in  the  peripheral  nerves. 

The  clinical  manifestations  of  the  disease  consist  of 
abnormal  sensations  of  pain  and  of  paresthesia,  impairment 
or  loss  of  various  forms  of  sensibility,  diminution  of  muscular 
tone,  loss  of  the  deep  reflexes,  sphincter  troubles,  inco-ordina- 
tion,  trophic  disturbances,  and  visceral  complications. 

Etiology.  The  disease,  except  for  a  special  type  occurring 
in  the  young  and  known  as  juvenile  tabes,  is  one  of  early 
adult  and  middle  life.  About  50  per  cent,  of  the  cases  com- 
mence between  the  ages  of  thirty  and  forty.  It  affects  men 
more  frequently  than  women,  the  relative  proportions  being 
about  ten  to  one. 

Syphilis  is  the  most  important,  and,  indeed,  the  funda- 
mental factor  in  the  production  of  tabes.  Tabes  is  a  degene- 
rative process  of  syphilitic  origin — a  parasyphilitic  disease. 
The  arguments  urged  against  the  syphilitic  origin  of  tabes  are 
based  chiefly  upon  the  absence  of  a  syphilitic  history  in  a 
small  proportion  of  cases,  and  upon  the  frequent  failure  of  the 
antisyphilitic  remedies  in  the  treatment  of  the  malady. 

Our  statistics  show  the  large  proportion  of  the  cases  of 
tabes  and  general  paralysis  in  which  a  history  of  venereal 
infection  was  giveo.  Out  of  166  cases,  140  gave  a  history 
of  previous  syrphilitic  or  gonorrhoeal  infection.  In  the  cases 
of  tabes  alone  76*6  per  cent,  gave  a  history  of  syphilitic 
infection,  a  percentage  which  was  raised  to  89*5  per  cent, 
if  gonorrhoeal  infection  was  included.  Of  the  paretics,  47*6 
per  cent,  admitted  to  syphilitic  infection,  a  percentage  which 
was  increased  to  69  per  cent,  when  other  forms  of  venereal 
infection  were  included. 

The  cases  in  which  venereal  infection  was  denied  formed 
a  relatively  small  proportion  of  the  total  number,  but  it  is 
interesting  to  note  that  in  the  cases  examined,  a  cytological 
examination  of  the  cerebro-spinal  fluid  gave  the  same  marked 
lymphocytosis  as  was  observed  in  the  definitely  syphilitic 
cases. 

Heredity  plays  quite  an  unimportant  part  in  the  pro- 
duction of  the  disease,  except  in  cases  of  juvenile  tabes,  where 
hereditary  syphilis  is  practically  universal. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    381 

Injuries,  acute  diseases,  severe  strain,  exposure  to  cold, 
and  alcoholic  and  sexual  excess  have  all  been  cited  as 
determinant  factors  in  the  production  of  the  disease,  and 
undoubtedly  play  a  part  by  lowering  the  vitality  of  the 
nervous  system. 

Morbid  anatomy.  The  naked-eye  examination  reveals 
some  thickening  of  the  pia  arachnoid  over  the  dorsal  surface 
of  the  spinal  cord,  which  is  often  somewhat  flattened.     The 


Fig.  113. — A  transverse  section  of  the  spinal  cord  from  a  case  of  early 
tabes  dorsalis. 


posterior  roots  are  thin,  but  the  atrophy  is  not  universal, 
though  often  symmetrical.  On  making  transverse  sections 
of  the  cord,  the  posterior  columns  are  found  to  be  shrunken 
and  discoloured,  their  dull  grey  translucent  appearance 
contrasting  with  the  whiteness  of  the  anterior  and  lateral 
columns.  These  changes  are  most  noticeable  in  the  lumbar 
region  and  the  corresponding  posterior  nerve  roots.  Degene- 
ration of  the  optic  and  some  other  cranial  nerves  may  also 
be  noted. 

The  characteristic  pathological  change  in  tabes  is  a 
degeneration  of  the  sensory  fibres,  which  enter  the  cord  from 
the  posterior  roots.  The  posterior  columns  are  composed 
of  exogenous  fibres  which  have  their  origin  in  the  posterior 
root  ganglia,  and  of  endogenous  fibres  which  arise  from  cells 
situated  within  the  spinal  cord.     As  already  mentioned,  the 


382 


XKKYOrs   DISEASES 


exogenous  fibres  are  those  which  are  chiefly  affected  in  tabes, 
and  certain  of  them  suffer  more  than  others. 

There  are  four  distinct  sets  of  fibres  which  pass  into  the 
cord  from  a  posterior  root.  (1)  Short  fibres,  which  form 
the  inner  bundle  of  the  posterior  root,  and,  after  running  a 
short  course  in  the  cord,  terminate  in  the  posterior  horn  of 


Figs.  114  AND  115. — Two  sections  of  the  Spina]  curd  from  a  case 
of  -well-marked  tabes  dorsalis. 


the  same  side.  (2)  Medium  fibres,  which  pass  up  for  some 
distance  in  the  posterior  columns,  and  terminate  by  giving 
off  collaterals  to  the  cells  of  Clarke's  column  and  to  the 
anterior  horn.  (3)  Long  fibres,  which  pass  up  the  posterior 
columns  to  the  posterior  nuclei  of  the  same  side.  As  these 
fibres  are  displaced  more  and  more  towards  the  mesial  septum 
by  those  coming  in  from  each  posterior  root,  it  comes  about 
that  the  postero-internal  column  in  the  cervical  region 
contains   the    fibres   which   have   entered    from    the    sacral, 


GENEEAL  DISEASES  OF  THE  NERVOUS  SYSTEM     383 

lumbar,  and  lower  dorsal  roots.  (4)  Fine  fibres,  which  pass 
into  the  zone  of  Lissauer,  and  form  a  cap  for  the  posterior 
horn. 

The  picture  of  degeneration  found  in  a  case  of  tabes, 
therefore,  depends  upon  the  stage  of  the  disease  at  which  the 
cord  is  examined.  The  fibres  which  degenerate  earliest  are : 
(1)  the  medium  fibres  and  their  collaterals ;  and  (2)  the  fine 
fibres  of  the  zone  of  Lissauer. 

The  medium  fibres  are  displaced  towards  the  middle  of  the 
posterior  columns,  and  lie  in  the  inner  part  of  the  postero- 
external division ;  so  that  if  the  case  be  an  early  one,  the 
degeneration  will  be  noticed  in  that  region,  where  the  tract 
has  received  the  name  of  'Bandelette  externe,'  as  well  as  in 
the  zone  of  Lissauer,  to  which  the  fine  fibres  pass.  If  the 
case  be  more  advanced,  degeneration  will  be  noticed  in 
the  long  and  short  fibres  as  well,  so  that  there  will  be  com- 
plete degeneration  of  the  postero-internal  column,  except  for 
the  endogenous  fibres.  If  the  disease  is  mainly  limited  to  the 
lumbo-sacral  region,  degeneration  will  be  found  in  the  postero- 
external columns  of  the  lower  lumbar  and  sacral  regions, 
and  in  the  postero-internal  columns  of  the  cervical  region. 
If  the  disease  is  chiefly  in  the  cervical  region  (cervical  tabes), 
degeneration  will  be  noted  in  the  postero-external  column 
of  the  cervical  region  only. 

The  lesion  consists  of  a  primary  degeneration  of  nerve 
fibres,  the  myeline  sheath  breaking  up,  and  the  fibre 
eventually  being  destroyed.  As  a  secondary  effect  prolifera- 
tion of  the  neuroglial  tissue  takes  place,  so  that  a  dense 
network  of  neuroglia  is  formed,  in  which  few  or  no  nerve 
fibres  can  be  distinguished. 

Thickening  of  the  pia  arachnoid  over  the  posterior  aspect 
of  the  spinal  cord  is  not  uncommon,  and  in  some  cases  there 
is  a  leptomeningitis. 

In  connexion  with  the  posterior  horns  there  is  observed 
degeneration  of  the  collateral  reflex  fibres  and  fibres  round 
Clarke's  column ;  the  cells  of  the  posterior  horn  show  no 
change. 

Degenerative  changes  in  the  anterior  horn  cells  of  varying 
degree  and  intensity  may  be  noted,  but  such  changes  are 
rare,  and  are  met  with  chiefly  in  those  cases  of  tabes  associ- 
ated with  muscular  atrophy.       The   anterior   horn    changes 


334  NERVOUS  DISEASES 

are,  in  all  probability,  secondary  to  changes  in  the  motor 
nerves. 

Posterior  nerve  roots.  In  early  cases  very  little  change 
may  be  made  out  in  the  posterior  nerve  roots  outside  the  cord, 
although  degeneration  of  the  exogenous  fibres  within  the  cord 
may  be  well  marked.  In  more  advanced  cases  the  myeline 
sheaths  of  the  fibres  swell,  disintegrate,  and  finally  disappear, 
especially  where  the  roots  pass  through  the  pia-arachnoid 
membrane.  This  degeneration  may  extend  in  extreme 
cases  into  the  posterior  root  ganglia.  Secondary  to  the 
parenchymatous  degeneration  of  the  myeline  sheaths,  there 
is  a  proliferation  of  the  surrounding  perineural  connective 
tissues. 

Posterior  root  ganglia.  Atrophic  changes  in  the  ganglion 
cells  and  secondary  proliferation  of  the  interstitial  tissues 
have  been  described  in  a  few  cases,  but  such  changes  are 
rare,  and  are  probably  only  secondary  to  the  degeneration  of 
the  posterior  roots.  They  are  certainly  not  accountable  for 
the  degeneration  of  the  posterior  root  fibres,  which  may 
be  extreme  in  degree,  without  any  obvious  change  in  the 
ganglia. 

Peripheral  nerves.  The  changes  found  in  the  peripheral 
nerves  are  generally  confined  to  the  small  sensory  fibres  near 
their  peripheral  distribution  in  the  skin,  and  are  never  in  any 
degree  comparable  to  the  degeneration  found  in  the  posterior 
root  fibres.  Degeneration  of  the  motor  nerve  fibres  of  the 
spinal  and  cranial  nerves  has  been  described  in  cases  with 
muscular  atrophy. 

Degeneration  of  the  optic  nerves  is  not  uncommon,  and 
commences  in  the  peripheral  portion  of  the  nerves  near  the 
disc  and  extends  towards  the  brain.  Degeneration  of  the 
ganglion  cells  in  the  retina  has  been  seen  in  some  cases,  but 
in  others,  where  there  was  well-marked  optic  atrophy,  no 
degeneration  was  found  in  the  retinal  cells.  The  degeneration 
is  first  noticed  where  the  fibres  lose  the  myeline  sheath  at 
their  entrance  into  the  eyeball ;  it  is  possible  that  this  is 
their  most  vulnerable  portion,  but  on  the  other  hand  there  is 
some  evidence  to  suggest  that  the  optic  nerve  atrophy  is  not 
a  true  tabetic  atrophy,  but  is  due  to  an  interstitial  neuritis 
with  secondary  pressure  and  atrophy  of  the  optic  fibres 
(Leri). 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    385 

The  sensory  roots  of  the  fifth  nerve  and  the  auditory  nerve 
have  been  found  degenerated  in  some  cases.  In  the  medulla 
degeneration  of  the  sensory  fibres  of  the  fifth,  the  solitary 
bundle  and  the  posterior  nucleus  of  the  vagus  have  been 
observed. 

Marina  has  described  atrophic  changes  in  the  cells  of  the 
ciliary  ganglion  in  cases  with  reflex  iridoplegia. 

The  cerebro- spinal  fluid.  In  tabes  a  considerable  increase 
of  the  lymphocytes,  up  to  100  or  150  or  more,  is  found  on 
microscopic  examination  of  the  centrifuged  deposit.  This 
lymphocytosis  is  useful  as  a  diagnostic  point  in  cases  where 
the  diagnosis  is  otherwise  uncertain. 

The  morbid  changes  occurring  in  the  tabetic  arthropathies 
consist  chiefly  of  distension  of  the  capsule  of  the  joint,  which 
may  eventually  atrophy.  The  synovial  membrane  becomes 
thin  and  shreddy,  the  cartilage  disappears,  and  the  bone  is 
eroded  and  destroyed.  In  other  cases  a  new  formation  of 
bone  develops  in  the  form  of  bony  outgrowths,  or  deposits 
around  the  joint. 

The  bones  of  the  limbs  sometimes  become  rarefied  and  are 
liable  to  spontaneous  fractures.  In  other  cases  a  syphilitic 
osteitis  or  sclerosis  is  observed. 

Blood-vessels.  Although  the  occurrence  of  gummata  is 
rare  in  tabes,  atheroma  and  chronic  aortitis  are  frequently 
found. 

Symptoms.  The  clinical  course  of  a  typical  case  of  loco- 
motor ataxy  has  been  divided  into  three  stages,  according  to 
the  predominance  of  certain  symptoms.  The  classical  division 
into  preataxic,  ataxic,  and  paralytic  stages,  although  con- 
venient for  description,  is  far  from  being  universally  applicable. 
Even  in  the  first  stage  some  degree  of  instability  and  inco- 
ordination of  movement  may  be  detected  by  careful  examina- 
tion, and  it  is  only  when  the  inco-ordination  becomes  obtrusive 
that  the  second  stage  may  be  said  to  appear ;  and  the  third 
or  so-called  paralytic  stage  is  merely  one  of  overwhelming 
ataxia.  It  is  therefore  obvious  that  these  divisions  are 
entirely  arbitrary  and  are  based  solely  upon  the  clinical  mani- 
festations of  one  symptom,  ataxia.  Clinical  experience  daily 
demonstrates  the  fallacy  of  this  classification.  We  have  seen 
cases  in  which  the  initial  symptom  was  acute  ataxy  with- 
out subjective  sensory  symptoms  or  sphincter  disturbances ; 


386  NKRVOUS  DISEASES 

moreover,  many  cases  which  come  under  treatment  in  the 
ataxic  stage  recover  from  their  ataxy. 

As  the  mode  of  onset  and  course  of  the  disease  vary  so 
widely,  the  symptoms  are  best  considered  seriatim,  as  they 
affect  the  several  systems. 

The  mental  condition  of  tabetics  should  be  carefully  ex- 
amined, in  view  of  the  close  association  which  exists  between 
tabes  and  general  paralysis  of  the  insane.  In  uncomplicated 
cases  the  mental  condition  remains  normal,  but  in  a  few 
instances  delusions  of  persecution,  melancholia,  and  occa- 
sionally suicidal  tendencies  are  present. 

In  some  cases  mental  symptoms  characteristic  of  general 
paralysis  gradually  develop,  but  in  others  the  onset  of 
tabes  and  the  mental  symptoms  of  paretic  dementia  occur 
simultaneously  (tabo-paresis) . 

Cranial  nerves.  Degenerative  changes  in  the  olfactory, 
glossopharyngeal,  and  auditory  nerves,  with  clinical  evidence 
of  loss  of  function,  have  been  found  in  a  few  cases. 

Vision.  Progressive  loss  of  sight,  with  contraction  of  the 
visual  fields,  occurs  in  about  10  per  cent,  of  the  cases  of  tabes. 
It  is  due  to  atrophy  and  degeneration  of  the  optic  nerves. 
Tabetic  optic  atrophy  as  a  rule  results  in  total  failure  of 
vision.  Optic  atrophy  may  be  an  early  or  a  late  symptom, 
and  affects  one  eye  first,  but  its  development  has  no  constant 
influence  on  the  progress  of  the  disease. 

The  pupils.  Large  pupils  are  sometimes  met  with,  and 
when  present  are  often  associated  with  paralysis  of  the  third 
nerve  or  with  optic  atrophy.  Small  pupils  are  also  observed, 
and  generally  in  association  with  cervical  tabes,  but  medium- 
sized  pupils  are  the  most  common. 

The  pupils  may  be  equal  in  size  or  exhibit  a  striking 
inequality ;  as  a  rule  careful  observation  will  show  some 
inequality.  Sometimes  the  position  of  the  pupil  is  eccentric. 
The  outline  of  the  pupil  is  rarely  circular  ;  it  usually  shows 
definite  irregularities,  which,  if  not  due  to  local  causes, 
constitute  a  sign  characteristic  of  tabes,  general  paralysis,  and 
cerebro-spinal  syphilis.  The  reaction  of  the  pupils  to  con- 
vergence and  accommodation  is  generally  preserved,  although 
in  a  small  number  of  cases  it  is  impaired  or  lost.  In  marked 
contrast  to  this  is  the  interference  with  the  light-reaction 
which  is  so  constant  a  feature.     Loss  of  the  light-reflex  of  the 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM     387 

pupil,  with  preservation  of  the  contraction  on  accommodation, 
is  known  as  the  Argyll-Robertson  phenomenon,  and  is  found 
in  about  two-thirds  of  the  cases  of  tabes.  Loss  of  the 
pupillary  light-reaction  may  be  complete  or  incomplete,  or 
it  may  be  unilateral  or  bilateral.  The  contraction  may  be 
feeble  and  sluggish  and  followed  by  a  rapid  dilatation,  or  the 


Fig. 


116. — Illustrates  the  characteristic  appearance  of  the  face  in  a 
case  of  old-standing  tabes  dorsalis — the  tabetic  face. 


pupil  may  oscillate  (hippus).  Although  the  direct  pupillary 
light-reaction  is  lost  in  one  eye,  yet  the  consensual  light- 
reaction,  when  light  is  thrown  upon  the  other  eye,  may  be 
unimpaired. 

The  practical  outcome  of  these  observations  is  that  any 
interference  with  the  pupillary  light-reflex,  associated  with 
irregularity  of  the  contour  of  the  pupil  (not  due  to  local 
causes),  is  as  significant  a  sign  of  tabes  as  the  complete  loss 
of  the  light -reflex. 

25  * 


388  NER  Vol's  DISEASES 

The  reaction  of  the  pupils  to  stimulation  of  the  skin  of 
the  neck  may  also  be  lost  or  impaired. 

External  ocular  muscles.  Temporary  paralysis  or  paresis 
of  an  external  ocular  muscle  is  not  uncommon  in  the 
early  stages,  and  a  history  of  temporary  diplopia,  or  ptosis, 
is  often  obtained.  Permanent  paralysis,  or  paresis,  of  one 
sixth  nerve  or  of  one  third  nerve  is  frequently  observed. 
Temporary  paralysis  of  the  third  nerve  always  results  in  a 
permanent  loss  of  the  pupillary  light-reflex.  Although  these 
paralyses  frequently  occur,  the  most  common  affection  of  the 
ocular  nerves  is  a  slight  drooping  of  both  upper  lids, 
with  compensatory  over-action  of  the  frontalis  muscles. 
This  feature,  in  conjunction  with  small  pupils  which  give 
the  eyes  a  steel-like  look,  constitute  the  '  tabetic  facies.' 
(Fig."  116.) 

The  motor  functions  of  the  fifth  nerve  are  rarely  affected, 
but  the  sensory  portion  sometimes  suffers.  This  gives  rise  to 
lancinating  pains  and  occasionally  loss  of  sensibility  in  the 
distribution  of  the  nerve. 

The  seventh  nerve  is  not  affected. 

Affections  of  the  palate,  pharynx,  and  larynx  are  frequent. 
These  may  be  either  sensory,  such  as  loss  of  taste,  anaesthesia 
and  paresthesia,  or  motor  abductor  paralysis  of  the  vocal 
cords.  These  are  particularly  common,  occurring  in  about 
40  per  cent,  of  the  cases.  Unilateral  or  bilateral  paralysis 
of  the  abductors  of  the  vocal  cords  is  chief  amongst  them  ; 
and  although  it  gives  rise  to  no  phonetic  loss,  dyspnoea  and 
discomfort  may  be  present.  Paralysis  of  the  adductors  of  the 
cords  and  of  the  soft  palate  is  rare. 

Unilateral  atrophy  of  the  tongue  occurs  sufficiently  often 
to  be  mentioned  as  an  occasional  occurrence. 

Motor  system.  The  motor  system  suffers  indirectly 
as  the  result  of  the  affection  of  the  sensory  apparatus.  The 
muscles  may  exhibit  some  signs  of  general  malnutrition 
and  reduction  in  size,  but  as  a  rule  local  wasting  does  not 
occur.  There  are,  however,  a  not  inconsiderable  number  of 
cases  in  which  muscular  atrophy  is  associated  with  tabes,  and 
in  these  cases  changes  in  the  motor  nerves,  or  in  the  anterior 
horn  cells,  or  their  bulbar  analogues,  have  been  found  to 
account  for  the  wasting. 

The  muscular  irritability  to  mechanical  stimulation  may 


GENEKAL  DISEASES  OF  THE  NERVOUS  SYSTEM    389 


be  diminished  in  the  later  stages  of  the  disease.  The  power 
of  individual  muscles  is  well  maintained,  although  a  slight 
relative  loss  of  power  is  common,  and  depends  upon  the 
degree  of  muscular  inco-ordination. 

Atonia.  The  most  characteristic  change  in  the  motor 
system  is  the  impairment  or  loss  of  tone  in  the  muscles — 
hypotonia  and  atonia.  It  is  due  to  the  loss  of  the  sensory 
stimuli  impinging  upon  the 
motor  centres  in  the  spinal 
cord.  It  is  not  confined  to 
the  muscles,  but  involves  the 
ligaments  in  and  around  the 
joints.  It  is  shown  by  flacciclity 
of  the  muscles,  by  the  diminu- 
tion or  loss  of  the  deep  reflexes, 
and  in  severe  cases  by  an 
abnormal  degree  of  movement 
at  the  several  joints.  Hyper- 
extension  of  the  knee  joint 
(genu  recurvatum)  and  abnor- 
mal flexion  of  the  hips,  whereby 
a  patient  may  be  enabled  to 
place  both  his  feet  behind 
his  head,  are  examples  of  the 
effect  of  this  loss  of  ligamen- 
tous and  muscular  tone.  (Figs. 
117  and  118.) 

Ataxia.  The  inco-ordination 
of  muscular  action  gives  rise  to  ataxia.  It  may  be  an  early, 
but  is  more  usually  a  late,  symptom.  Its  onset  is  as  a  rule 
gradual,  but  sometimes  extremely  rapid.  When  slight  in 
degree  the  patient  may  obviate  it  by  using  his  visual  impres- 
sions to  guide  his  erring  limbs.  Thus  it  is  that  the  earliest 
symptoms  are  noticed  when  he  is  deprived  of  vision,  as  when 
in  the  dark  or  when  the  eyes  are  closed.  Ataxy  is  due  to 
the  loss  of  the  sense  of  position  in  the  muscles  and  the 
joints.  It  gives  rise  to  an  inability  to  perform  the  finer 
movements  of  the  fingers  and  the  hands,  such  as  buttoning 
the  clothes  and  writing,  and  to  an  inability  to  walk  along  a 
straight  line.  In  the  earlier  stages  closure  of  the  eyes  may 
reveal  inco-ordination  in  the  performance  of  the  finger-to-nose 


Fig.  117.  —  Photograph  showing 
genu  recurvatiini  or  hyperexten- 
sion  of  the  knee  joint. 


390  NEK  Vol's  DISEASES 

test,  or  increased  unsteadiness  on  standing  with  the  feet 
together  (Romberg's  sign).  In  advanced  cases  the  patient 
cannot  walk,  and  sometimes  cannot  even  feed  himself. 

Gait.  In  typical  cases  the  gait  is  unsteady  and  stagger- 
ing, the  patient  walks  with  his  head  bent  down  and  his  eyes 
fixed  upon  the  ground.  His  feet  are  everted  and  he  walks 
upon  a  wide  base ;  the  movements  of  his  legs  are  excessive, 
the  feet  are  thrown  out  too  high,  and  the  heels  come  down 
sharply  on  the  ground.     Any  attempt  to  stop  suddenly,  or  to 


Fig.  118. — Illustrates  an  extreme  degree  of  hypotonia. 

turn  round,  results  in  much  staggering,  and  perhaps  a  fall. 
Darkness  increases  the  difficulty  in  walking  or  may  render  it 
impossible. 

Sensory  system.  The  sensory  symptoms  of  tabes  are 
both  subjective  and  objective.  The  most  characteristic  of  the 
subjective  symptoms  are :  (a)  Sharp,  shooting,  lancinating 
pains,  which  occur  in  paroxysms  commencing  suddenly  and 
terminating  abruptly.  The  pains  are  felt  in  the  fleshy  parts 
immediately  under  the  surface  of  the  skin,  and  are  specially 
frequent  in  the  legs  and  ulnar  side  of  the  forearm.  Similar 
pains  often  radiate  from  and  to  the  joints.  After  a  bout  of 
such  pains  there  is  often  extreme  hyperesthesia  and  tender- 
ness to  tactile  impressions  on  the  skin  over  the  affected  area. 
(b)  Deep  pains  of  a  gnawing  and  burning  character,  which 
may  last  for  days  or  weeks,  and  are  usually  referred  to  the 
bones  or  deep  muscles,  (c)  Superficial  tenderness  and  feelings 
of  constriction  by  hot  or  painful  bands,  of  which  the  girdle 
sensation  is  the  most  common  example.  Besides  these,  a 
sense  of  compression,  numbness,  and  tingling  are  commonly 
described. 

Such  subjective  phenomena  are   usually  early    symptoms 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    391 

of  the  disease  and   are  often  attributed  to  rheumatism  or 
gout. 

The  objective  examination  of  the  several  forms  of 
cutaneous  and  deep  sensibility  reveal  many  characteristic 
abnormalities. 

1.  The  loss  of  sense  of  position  is  seen  in  the  inco-ordina- 
tion  and  unsteadiness  to  which  it  gives  rise.  As  a  rule,  the 
distal  parts  of  the  limbs  suffer  before  the  proximal.  Thus,  a 
patient  may  be  unable  to  tell  in  what  position  his  toes,  feet, 
or  legs  are  placed,  and  when  asked  to  point  to  his  foot 
will  take  as  a  guide  the  impressions  received  from  the  hip 
joint.  Similarly  in  the  upper  limb  he  may  not  know  the 
position  of  his  fingers,  but  be  able  to  tell  the  position  of  his 
hand  and  forearm. 

2.  The  loss  of  deep  pain  (painful  pressure)  is  an  early 
symptom,  especially  in  the  muscles  of  the  legs,  when  painful 
pressure  on  the  calf  muscles  is  not  appreciated  as  such.  The 
loss  of  deep  pain  is  a  valuable  sign  in  the  differential  diagnosis 
of  tabes  from  alcoholic  neuritis. 

3.  Cutaneous  pain  is  usually  impaired  out  of  proportion  to 
the  loss  of  tactile  sensibility.  In  the  early  stages  it  may  be 
diminished  or  delayed,  in  advanced  cases  it  is  lost. 

4.  Extremes  of  heat  and  cold  are  impaired  to  much  the 
same  degree  as  cutaneous  pain,  and  both  are  usually  affected 
over  the  same  areas. 

5.  Discrimination  and  vibration  are  often  impaired,  but 
as  a  rule  later  in  the  course  of  the  disease  and  less  completely 
than  those  already  mentioned. 

6.  The  sensibility  to  moderate  degrees  of  temperature  and 
light  tactile  sensibility  are  frequently  found  to  be  slightly 
defective,  but  such  changes  are  late  in  appearing.  Its  pre- 
servation is  in  marked  contrast  to  the  loss  of  painful 
sensibility. 

7.  Contact  and  pressure  are  preserved  in  almost  all  cases, 
and  are  the  last  to  disappear. 

The  distribution  of  the  sensory  changes  depends  entirely 
on  the  situation  of  the  disease.  In  cases  where  the  lumbo- 
sacral roots  are  chiefly  involved,  the  sensory  loss  is  confined  to 
the  perinseum  and  lower  limbs.  In  cervical  and  dorsal  tabes 
the  ulnar  border  of  the  arm  and  the  upper  thoracic  root  areas 
are  chiefly  affected.     (Fig.  119) 


392 


NERVOUS  DISEASES 


But  as  in  most  cases  of  tabes  both  the  cervical  and  lumbo- 
sacral regions  of  the  spinal  cord  are  the  seat  of  degeneration, 
characteristic  sensory  disturbances  may  be  demonstrated  over 
both  the  upper  and  lower  limbs  and  the  trunk.  In  rare  cases 
the  sensory  loss  is  found  in  the  distribution  of  the  fifth  nerve. 
Reflexes.  Interference  with  the  plantar,  bulbo-cavernous, 
cremasteric,  and  anal  reflexes  is  common.     These  reflexes  are 

affected  pari  passu  with  the  involve- 
ment of  the  lumbo-sacral  cord.  The 
epigastric  and  abdominal  reflexes  are 
rarely  lost>  as  the  dorsal  roots  are 
often  not  involved  in  the  tabetic  pro- 
cess. In  cases  of  gastric  crises  the 
superficial  epigastric  reflexes  may 
be  hypersensitive. 

Deep  reflexes.  These  are  at  first 
diminished  and  ultimately  lost.  If 
the  disease  commences  in  the  lumbo- 
sacral region  the  ankle  and  knee  jerks 
disappear,  as  a  rule  the  ankle  before 
the  knee  jerk.  The  deep  reflexes  of 
the  upper  limbs  are  not  lost  unless 
the  upper  cord  is  affected.  In 
advanced  cases  all  the  deep  reflexes, 
including  the  jaw  jerk,  may  be 
abolished. 

Organic  reflexes.  Interference 
with  micturition  may  be  an  early 
sign.  At  first  there  is  '  stammer- 
ing '  micturition,  by  which  is  meant 
that  the  patient  feels  an  urgent  call  to  pass  water,  but 
when  he  tries  to  do  so,  fails,  but  having  failed  he  may 
shortly  after  have  precipitant  micturition,  which  he  is 
unable  to  control.  Later  there  may  be  loss  of  control  at 
night,  and  when  excited  during  the  day.  The  final  stage 
is  over-distension  of  the  bladder,  leading  to  dilatation  of  the 
ureters  and  pelvis  of  the  kidney. 

Distension  of  the  bladder  may  occur  quite  unknown  to  the 
patient,  as  owing  to  the  loss  of  sensibility  he  has  no  feeling  of 
pain  or  discomfort.  The  liability  of  the  residual  urine  to 
become  septic  and  give  rise  to  cystitis  and  pyonephrosis  con- 


Pig.  111*. — Chart  showing  a 

common  distribution  of 
the  sensory  impairment 
in  tabes.  Dots  signif] 
subjective,  lines  objec- 
tive phenomena. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    393 

stitutes  one  of  the  most  serious  complications  of  the  disease, 
and  is  in  many  cases  the  ultimate  cause  of  death.  Constipa- 
tion is  common,  but  in  some  cases  attacks  of  diarrhoea  with 
loss  of  control  over  the  anal  sphincter  occur. 

Generative  functions.  In  the  early  stage  the  patient 
may  complain  of  pains  in  the  genital  organs  with  an  increase 
of  the  sexual  desire.  Erections  and  nocturnal  emissions  are 
frequent.  In  the  later  stages  sexual  power  and  desire  are  lost 
and  erections  cannot  be  obtained.  This  is  often  associated 
with  loss  of  testicular  sensation  and  atrophy  of  the  testicles. 

Visceral  crises.  The  commonest  is  the  gastric  crisis. 
This  consists  of  attacks  of  nausea  and  vomiting,  of  sudden 
onset,  accompanied,  or  preceded,  by  severe  pains  in  the  region 
of  the  stomach  passing  round  the  body  or  through  to  the  back. 
The  skin  over  the  epigastric  region  may  become  hypersesthetic. 
Vomiting  is  persistent,  and  the  patient  brings  up  what  food 
he  may  have  in  his  stomach,  then  bile  and  blood.  The  attacks 
may  last  for  a  few  hours  or  several  days.  They  occur  during 
the  early  stages  of  the  disease,  and  as  a  rule  tend  to  become 
less  severe  and  less  frequent  as  years  go  on.  The  ingestion 
of  food  is  not  an  exciting  cause  in  the  majority  of  cases,  but 
in  some  a  disordered  stomach  undoubtedly  gives  rise  to  an 
attack. 

Laryngeal  crises  are  of  two  types.  The  mild  are  charac- 
terised by  an  obstinate  dry  cough,  occurring  at  frequent 
intervals,  and  ending  by  the  expectoration  of  a  quantity  of 
viscid  mucus ;  the  severe  by  pain,  stridor,  and  a  feeling  of 
suffocation  and  impending  death,  the  lips  become  livid  and 
the  skin  pale,  and  there  is  indrawing  of  the  chest  and  sub- 
sternal region.  These  attacks  pass  off  as  suddenly  as  they 
come  on. 

Laryngeal  crises  may  be  present  when  there  is  no  paralysis 
of  the  vocal  cords,  or  both  may  be  combined.  Paralysis  may 
exist  without  laryngeal  crises,  but  often  follows  such  attacks. 

Among  the  rarer  forms  of  visceral  crises  are  rectal, 
nephritic,  intestinal,  cardiac,  and  respiratory  crises. 

Trophic  changes.  The  more  serious  trophic  changes  which 
occur  in  tabes  are  (1)  ai'thropathies,  and  (2)  trophic  ulcers 
and  sores.  The  changes  in  the  joints  are  as  a  rule  of  gradual 
onset,  and  are  often  preceded  by  pains  in  and  around  the 
affected  joint.     The  first  stage  in  the  process  consists  of  a 


WM 


NERVOUS  DISEASES 


relaxation  of  the  ligaments  of  the  joint ;  later,  the  synovial 
membranes  become  thickened  and  ossification  takes  place  in 
the  fringes.  The  bones  maj'  either  hypertrophy  or  atrophy, 
and  the  joint  becomes  distended  with  synovial  fluid.  This 
results  in  the  total  disorganisation  of  the  joint.  Various 
complications  may  arise,  such  as  a  serous  or  hemorrhagic 
effusion  into  the  joint,  or  the  limb  below  the  joint  may  become 


Fig.   120. — Illustrates  arthropathy  (Charcot's  joints)  of  the  right 

knee  and  ankle  in  the  case  of  tabes. 


the  seat  of  a  hard  and  tense  cedema.  Secondary  to  the 
joint  changes  the  surrounding  muscles  undergo  atrophy,  and 
in  some  cases  the  peripheral  nerves  may  be  pressed  upon 
with  resulting  paralysis.  These  changes  do  not  cause  the 
patient  any  pain,  and  suppuration  or  tuberculous  infection 
rarely  occur.  The  affection  is  usually  limited  to  one  joint. 
The  knee  joint  is  that  most  commonly  affected,  and  then, 
in  order  of  frequency,  the  hip,  shoulder,  ankle,  and  elbow. 
Hypertrophy  is  common  in  the  knee  and  elbow  joints,  and 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    395 

atrophy   when    the    hip    joint   is   the    seat    of   the    lesion. 
(Fig.  120.) 

Commonly  the  bones  become  rarefied  with  loss  of  osseous 
tissue,  and  in  such  cases  spontaneous  fractures  are  liable  to 
occur.  These  are  of  sudden  onset  without  any  exciting  cause, 
or  are  due  to  a  trivial  injury.  There  is  no  pain,  and  crepitus 
can  be  produced  without  the  patient's  knowledge.  The  union 
is  often  delayed  and 
an  abnormal  amount 
of  callus  is  thrown 
out.  The  bones  of  the 
lower  limbs  appear  to 
be  most  liable  to  spon- 
taneous fractures. 

The  'tabetic  foot' 
is  the  result  of  trophic 
changes  in  the  liga- 
ments, whereby  the 
arch  of  the  foot  sinks, 
the  foot  is  everted  and 
notched,  so  that  the 
patient  walks  upon  its 
inner  side  and  the 
metatarso  -  phalangeal 
joint  of  the  big  toe 
forms  a  prominence. 

Perforating  ulcers. 
These  ulcers  form  one 
of  the  special  signs  of 
tabes.  They  most 
frequently  arise  on  the~ 
plantar  surface  at  the 

i  *  n  I  -,       Fig.  121. — Illustrates    the    characteristic    per- 

base   Of  the    great   and  forating  ulcers  of  tabes. 

little  toes.  They  com- 
mence usually  as  a  corn,  round  which  the  patient  complains 
of  sharp  shooting  pains.  The  corn  becomes  excoriated,  and 
a  small  circular  ulcer  with  irregular  edges  is  formed.  From 
the  ulcer  a  serous  or  sero-purulent  discharge  exudes.  The 
ulcer  extends  deeply  so  as  to  expose  the  subjacent  bone,  of 
which  a  sequestrum  is  formed.  The  ulcer  itself  is  painless. 
(Fig.  121.) 


396  NERVOUS   DISEASES 

Similar  trophic  changes  may  occur  in  the  nose,  palate, 
and  jaws,  in  the  last  instance  resulting  in  the  loosening  and 
dropping  out  of  the  teeth. 

Barer  changes  are  spontaneous  rupture  of  tendons,  herpes 
of  the  skin,  subungual  hemorrhages,  purpura,  and  oedema. 
Falling  out  of  the  hair  and  brittleness  of  the  nails  are  other 
common  trophic  manifestations  of  the  disease. 

Emaciation,  sometimes  extreme,  is  an  almost  constant 
accompaniment  of  locomotor  ataxy.  It  occurs  in  all  types 
of  the  disease,  independently  of  the  amount  of  nourishment 
which  the  patient  is  taking. 

Clinical  types.  In  the  early  stages  the  disease  may  present 
several  types. 

1.  A  type  commencing  with  slight  limb  pains,  which  are 
usually  attributed  to  rheumatism,  and  which  may  persist  for 
many  years  before  the  onset  of  ataxia.  This  maybe  described 
as  a  type  with  a  prolonged  first  stage.  As  a  rule,  the  usual 
physical  signs  in  the  pupils  and  deep  reflexes  are  definite  even 
in  the  early  stages. 

2.  The  neuralgic  type.  This  is  a  rare  variety  characterised 
by  the  existence  of  severe  and  long-continued  pains,  often 
with  little  or  no  ataxy  or  sphincter  trouble,  and  with  preser- 
vation of  the  deep  reflexes.  The  pupil  changes  may  be  quite 
definite. 

3.  The  gastric  t}Tpe  is  characterised  by  the  frequent  recur- 
rence of  severe  gastric  crises.  Sensory  changes  and  ataxy 
may  be  absent  or  slight,  but  the  other  signs  of  the  disease  are 
usually  present. 

4.  The  trophic  type.  In  the  cases  of  this  type  joint 
changes  and  perforating  ulcers  are  the  early  symptoms,  and 
sometimes  persist  for  long  periods  before  any  other  symptoms 
develop.     This  type  is  often  associated  with  visceral  crises. 

5.  The  ataxic  t}'pe.  This  is  characterised  by  the  sudden 
onset  of  severe  ataxy,  often  preceding  the  advent  of  pains 
and  other  symptoms. 

6.  The  perineal  type.  In  this  the  action  of  the  vesical 
and  rectal  sphincters  is  impaired  and  the  sexual  functions  are 
abolished.  These  disturbances  may  precede,  or  be  accom- 
panied by  limb  pains,  but  are  rarely  associated  with  ataxy  in 
the  early  stages. 

7.  The  amblyopic  type.     In   this   type   loss  of  vision  and 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM     397 

optic   atrophy  may  be  found  in   association  with  loss  of  the 
deep  reflexes,  but  without  the  other  symptoms  of  the  disease. 

Prognosis.  The  prognosis  as  regards  life  is  good,  though 
the  disease  may  persist  for  many  years.  Its  average  duration 
is  about  twelve  or  fifteen  years.  Although  the  malady 
is  known  as  progressive  locomotor  ataxy,  it  is  a  matter  of 
practical  experience  that  care  and  judicious  treatment  may  do 
much  to  mitigate  the  symptoms  and  prolong  life.  In  many 
cases,  also,  it  would  appear  as  if  the  disease  became  arrested. 

The  following  complications  are  unfavourable :  (a)  Cases 
which  develop  symptoms  of  general  paralysis.  These  do 
not  survive  the  onset  of  the  mental  symptoms  more  than 
two  or  three  years,  (b)  Cases  complicated  with  vascular 
lesions,  either  general  arterial  degeneration,  or  aortic  insuf- 
ficiency with  aortitis  and  angina  pectoris.  (c)  Cases  with 
pronounced  bladder  complications,  especially  retention  of 
urine,  owing  to  the  risk  of  infection  of  the  urinary  passages. 

Cases  characterised  by  the  rapid  onset  of  ataxia,  even  when 
most  severe,  yield  in  a  remarkable  manner  to  the  influence 
of  rest  and  perseverance  with  co-ordinating,  or  Frrenkel's 
exercises. 

The  pains  are  difficult  to  allay,  but  even  their  continuance 
in  a  severe  degree  over  prolonged  periods,  although  temporarily 
exhausting,  do  not  interfere  with  the  duration  of  life,  or  modify 
the  course  of  the  disease. 

Treatment.  Much  may  be  done  in  this  disease  by  judicious 
care  and  treatment.  The  disease  would  appear  to  become 
arrested  in  some  cases,  and  troublesome  complications,  ter- 
minating in  death,  may  be  averted  or  controlled.  It  is  therefore 
essential  that  the  malady  should  be  taken  in  hand  as  soon  as 
its  nature  has  been  recognised,  and  the  patient  encouraged  by 
every  available  means  to  persevere  with  treatment. 

The  first  question  which  demands  consideration  is  whether 
antisyphilitic  treatment  should  be  adopted,  and  if  so,  what  is 
the  most  suitable  form  in  which  it  should  be  given.  To 
prescribe  a  course  of  this  treatment  in  every  case  of  tabes  is 
not  good  practice,  although  many  will  derive  benefit  from  it. 
Those  most  likely  to  be  improved  are  cases  in  which  the 
tabetic  symptoms  have  arisen  within  a  comparatively  short 
time  (five  years)  after  infection,  and  those  who  have  not 
previously  had  an  energetic  course  of  treatment  for  the  original 


398  NERVOUS  DISEASES 

syphilitic  infection.  Although  these  may  be  laid  down  as 
guiding  principles,  cases  of  tabes  which  do  not  fall  into  either 
category  may  with  advantage  undergo  a  course  of  mercury 
and  the  iodides.  In  cases  with  much  debility,  and  in  old- 
standing  cases,  general  tonic  treatment  will  be  found  of 
greater  service. 

Antisyphilitic  treatment  may  be  given  in  the  form  of  mer- 
curial inunction,  or  intramuscular  injections  in  combination 
with  the  iodides  by  the  mouth  (p.  378). 

General  treatment.  The  patient's  life  should  be  so  regu- 
lated that  all  excesses  are  avoided,  both  as  regards  work  and 
physical  exercise,  and  the  use  of  alcohol.  Exposure  to  cold 
and  wet  is  especially  detrimental,  so  that  residence  in  an 
equable  and  genial  climate  is  to  be  prescribed.  Equability  of 
climate  is  not  always  to  be  found  by  going  abroad,  and  we  have 
known  tabetics  whose  pains  have  been  less  and  whose  general 
comfort  has  been  greatest  when  resident  in  London.  An 
occasional  course  of  massage,  especially  if  given  in  combination 
with  mild  faradism,  and  for  a  time  rest  in  bed,  are  usually 
methods  which  may  be  adopted  when  there  is  any  temporary 
increase  in  the  symptoms,  such  as  pains,  marked  debility,  or 
rapidly  progressing  ataxia. 

Symptomatic  treatment.  Pains.  These  are  best  treated  by 
analgesics,  of  which  a  large  selection  is  at  the  physician's 
disposal — such  are  antipyrin,  acetanilide,  phenacetin,  aspirin, 
phenalgin,  exalgine,  pyramidon,  and  aluminium  chloride.  They 
may  be  prescribed  alone,  in  combination,  or  with  iodides  or 
salicylate  of  soda.  Morphia  is  now  rarely  needful,  but  if 
prescribed  should  be  given  hypodermically. 

Gastric  crises.  In  severe  attacks  no  drug  other  than 
morphia  will  give  relief.  In  milder  attacks,  codeia,  cocain, 
or  belladonna,  if  given  early,  may  sometimes  arrest  or  modify 
a  crisis.  A  useful  remedy  is  1  minim  of  tr.  iodi  in  a  wine- 
glass of  water  frequently  administered.  Small  quantities  of 
ice  to  suck  and  local  fomentations  will  soothe  the  discomfort 
and  pain  in  the  epigastric  region.  Occasionally  drinking  large 
quantities  of  water  is  successful.  Best  in  bed  is  usually 
necessaiy,  as  the  subsequent  prostration  is  often  severe. 

Bladder  weakness.  The  catheter  should  be  used  in  all 
cases  of  retention,  or  when  residual  urine  is  shown  to  be 
present.     Cystitis   may  be   relieved  either   by  urotropin,  or 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    399 

preferably  by  washing  out  the  bladder.  The  patient  should 
be  instructed  to  pass  his  urine  at  regular  intervals. 

Ataxia.  The  most  satisfactory  method  of  treating  this 
symptom,  is  that  introduced  by  Fnenkel.  The  principle  of 
his  method  lies  in  the  precise  performance  of  various  move- 
ments. This  precision  is  only  obtained  by  careful  repetition, 
the  patient  devoting  his  whole  attention  to  mastering  the 
exercises.  At  first  progress  is  slow  as  mental  and  bodily 
fatigue  are  induced.  As  benefit  only  results  from  the  proper 
appreciation  of  the  sensations  evoked  by  the  correct  execution 
of  the  movements,  the  exercises  should  not  be  continued  for 
longer  than  the  patient  is  able  to  sustain  his  attention.  By 
this  repetition  and  by  aid  of  what  sensation  remains,  the  patient 
learns  to  effect  his  movements  more  or  less  correctly.  In 
cases  where  vision  is  retained,  he  is  enabled  to  correct  his 
errors  and  to  attain  to  greater  proficiency ;  but  at  the  same 
time  he  must  note  the  sensations  coming  from  the  limb  which 
is  being  exercised.  By  the  full  appreciation  of  these  sensations 
he  is  eventually  able  to  dispense  with  the  aid  of  vision. 

In  severe  cases  the  patient  is  kept  in  bed  and  prescribed 
exercises,  which  he  can  carry  out  in  the  recumbent  posture. 
As  he  improves  he  is  taught  exercises,  first  in  a  sitting  position, 
then  when  standing,  and  later  on  when  walking  on  the  level, 
and  finally  up  and  down  stairs.  For  the  hands  and  arms 
exercises  at  a  dummy  keyboard,  or  a  '  solitaire '  board,  or 
writing  are  useful. 

The  treatment  should  be  continued  for  twelve  months  or 
more,  according  to  the  progress  made  by  each  case.  The 
duration  of  the  exercises  should  at  first  be  short — about  ten 
minutes  at  a  time  thrice  daily ;  but  later  on,  as  the  patient 
becomes  stronger  and  more  expert,  the  sittings  may  be 
prolonged  from  a  half  to  one  hour  three  times  daily.  They 
should  be  stopped  short  of  the  onset  of  fatigue. 

The  general  results  are  most  gratifying,  especially  in  young 
tabetics,  and  in  cases  of  pronounced  and  overwhelming  ataxy. 

Juvenile   tabes   dorsalis 

This  is  a  rare  condition,  and  affords  a  striking  example 
of  the  association  between  tabes  and  syphilis.  The  majority 
of  the  cases  are  due  to  inherited  syphilis,  although  a  small 


tOO  NERVOUS  DISEASES 

proportion  would  appear  to  arise  from  syphilis  acquired 
during  the  early  years  of  life.  In  some  cases,  although  no 
signs  of  congenital  syphilis  may  be  present  in  the  patient, 
a  parental  history  of  syphilis,  or  of  a  syphilitic  malady,  may 
be  obtained. 

The  symptoms  of  the  disease  are  essentially  similar  to  those 
seen  in  the  adult  forms.  The  symptoms  make  their  appear- 
ance shortty  before  or  at  puberty,  and  in  contradistinction  to 
the  greater  preponderance  of  the  adult  affection  in  the  male, 
the  juvenile  form  is  more  common  in  girls. 

There  are  two  chief  t}rpes:  one  in  which  the  symptoms 
commence  with  pains  in  the  limbs  and  ataxia  ;  the  other 
in  which  optic  atrophy  is  an  early  and  outstanding  feature. 
Argyll-Robertson  pupils,  sensory  disturbances,  and  loss  or 
diminution  of  the  deep  reflexes  are  invariably  present.  In 
many  instances  ataxia  is  absent  or  of  late  development.  In 
the  latter  type  evidences  of  congenital  syphilis  are  common. 

The  diagnosis  has  to  be  made  mainly  from  Friedreich's 
ataxy  (p.  471). 

The  prognosis  is  favourable  as  regards  the  life  of  the 
patient,  which  may  be  prolonged  for  many  years,  up  to 
seventeen  years  in  one  case. 


2.  PROGRESSIVE  GENERAL  PARALYSIS 
PARALYTIC    DEMENTIA 

General  paralysis  is  a  progressive  degenerative  disease  due 
to  decay  and  death  of  the  cortical  association  neurones  (Mott) 
characterised  by  mental,  motor,  and  sensory  symptoms. 

Etiology.  Statistics  show  that  from  75  to  85  per  cent, 
of  general  paralytics  have  suffered  from  syphilis.  The 
constant  relation  between  the  juvenile  form  of  general 
paralysis  and  congenital  syphilis  is  also  strong  proof  of  the 
causal  connexion  between  the  two  maladies.  The  not  infre- 
quent relation  and  coexistence  of  tabes  and  general  paralysis 
in  the  same  person  is  a  forcible  argument  not  only  in  favour 
of  the  essential  unity  of  the  two  diseases,  but  also  of  their 
common  origin  from  previous  syphilitic  infection.  In  this 
relation,  also,  a  point  has  been  made  of  the  inability  to  infect 
sufferers  from  general   paralysis    with  the   syphilitic  poison 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    401 

(Krafft-Ebing).  A  further  interesting  fact  in  favour  of  the 
syphilitic  causation  and  identity  of  tabes  and  general  para- 
lysis is  seen  in  the  cases  of  so-called  conjugal  tabes  and 
general  paralysis. 

Although  syphilis  is  the  predisposing  cause  of  the  malady 
other  factors  play  a  not  unimportant  part  in  its  production  : 
these  are  alcoholic  and  venereal  excess,  prolonged  mental  strain, 
worry,  anxiety,  and  overwork.  These  factors  may  induce 
symptoms  of  neurasthenia  in  healthy  individuals  in  middle 
life  as  well  as  in  those  who  in  youth  have  been  infected  by 
syphilis.  The  onset  of  neurasthenic  symptoms  after  thirty-five 
in  the  latter  should  not  be  treated  lightly,  and  should  call  for 
a  careful  examination.  Injury  to  the  head  in  cases  of  syphilis 
is  only  a  rare  cause  of  general  paralysis. 

Ford  Robertson l  and  others  have  stated  that  the  role  of 
syphilis,  in  the  causation  of  general  paralysis,  is  to  reduce  the 
immunity  of  the  nervous  system  to  bacterial  invasion  from 
other  parts  of  the  body. 

General  paralysis  is  more  common  in  men  than  in  women  ; 
in  the  latter  it  is  more  frequently  found  in  the  lower  social 
ranks.  It  is  more  common  also  in  urban  than  in  rural 
districts.  It  arises  more  often  between  thirty-five  and  fifty 
than  either  before  or  after  these  ages.  A  juvenile  form 
occurs  in  young  persons  during  puberty  or  early  adolescent 
life.  It  is  rarely  associated  with  any  family  predisposition 
to  mental  or  nervous  disease. 

Pathology.  To  the  naked  eye  the  brain  of  an  advanced 
case  of  general  paralysis  is  wasted  and  shrunken  ;  the  convo- 
lutions-particularly  of  the  frontal  and  Rolandic  areas — are 
atrophied,  and  the  pia  arachnoid  only  strips  off  with  difficulty: 
portions  of  the  subjacent  grey  cortex  coming  away  with  it. 
In  the  subdural  space  blood-clot  may  be  found  in  various 
stages  of  organisation  or  cystic  formation — the  condition 
known  as  pachymeningitis  hemorrhagica.  There  is  usually 
an  excess  of  cerebro-spinal  fluid  both  in  the  subdural  space 
and  in  the  ventricles,  the  ependyma  of  which  presents  a 
granular  appearance.  The  weight  of  the  brain  is  consider- 
ably  diminished. 

Microscopical    examination  of    the    cerebral  cortex   shows 

1  Ford  Robertson,  Review  of  Neurology,  1905 

26 


t02  NERVOUS  DISEASES 

degenerative  changes  in  both  the  vascular  and  nervous 
elements.  The  cortical  arterioles  present  thickening  of  the 
intima.  hyaline,  and  fatty  degeneration  of  the  muscular  coat, 
and  proliferation  of  the  perivascular  nuclei.  The  glial  cells 
are  large,  and  show  a  characteristic  'spider'  appearance;  while 
the  pyramidal  cells  show  various  stages  of  chromatolysis, 
degeneration,  and  atrophy.  Importance  has  been  placed  upon 
bhe  degeneration  of  the  tangential  and  association  fibres  of  the 
cortex,  more  particularly  of  the  frontal  lobe.  Similar  vascular 
and  nervous  lesions  are  found  in  other  parts — such  as  the 
basal  ganglia,  the  pons,  medulla,  and  spinal  cord. 

In  the  spinal  cord  secondary  degeneration,  both  in  the 
crossed  pyramidal  tracts  and  the  posterior  columnar  fibres, 
has  been  found.  The  optic  nerves  and  tracts  may  also  show 
a  degenerative  atrophy  similar  to  that  seen  in  tabes  dorsalis. 

The  fibres  of  the  peripheral  nerves  have  also  been  found 
degenerated  in  this  condition. 

The  cerebrospinal  fluid.  The  lymphocytes  are  greatly 
increased  in  number —  p  to  as  many  as  150  or  200  or  more 
c.mm.  of  fluid — and  the  Wasserman  reaction  is  present  in 
90  per  cent,  or  more  of  the  cases  (Mott).1 

Symptomatology.  The  symptomatology  of  general  para- 
lysis is  both  definite  and  characteristic.  Its  manifestations 
consist  of  mental  and  motor  phenomena.  The  earlier  and 
wider  recognition  of  its  many  types  has  to  some  extent  modified 
the  general  conception  of  its  early  stages  :  which  was  at  one 
time  based  upon  the  study  of  cases  seen  in  asylum  practice,  at 
a  period  distinctly  more  advanced  than  that  at  which  the 
disease  is  now  recognised  in  the  out-patient  department  of 
hospitals  and  in  private  practice. 

The  clinical  picture  of  the  fully  developed  disease  is 
characterised  by  mental  exaltation  with  grandiose  ideas, 
slurring  speech,  tremulous  lips  and  tongue,  and  pupillary 
changes. 

Mental  symptoms.  The  psychical  changes  consist  of: 
loss  of  memory  for  recent  events,  inability  to  concentrate 
attention,  impairment  of  the  finer  mental  faculties,  delu- 
sions, and  a  tendency  towards  progressive  dementia.  This 
dementia   may  commence  in  various  ways :    (a)    with   mild 

1  Mott,  Lancet,  1900. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    403 

exaltation,  grandiose  conceptions,  and  an  excessive  feeling 
of  well-being,  which  is  often  a  source  of  self-satisfaction  ; 
(b)  with  an  alteration  of  the  character,  so  that  the  patient 
commits  gross  acts  of  immorality  entirely  foreign  to  his 
nature ;  (c)  with  symptoms  of  neurasthenia,  associated  with 
insomnia,  mild  depression,  indecision  of  action,  and  some- 
times irritability  and  headache  ;  (d)  with  melancholia,  which 
presents  the  usual  features  of  depression,  delusions  as  to 
the  poisoning  of  food,  and  sometimes  suicidal  feelings  and 
homicidal  tendencies. 

In  whichever  way  the  dementia  starts,  the  tendency  is 
towards  a  progressive  increase  in  the  symptoms,  until 
eventually  a  condition  of  profound  dementia  results,  in  which 
memory  is  entirely  in  abeyance,  and  the  patient  pays  no 
attention  to  his  surroundings,  nor  to  the  care  of  his 
person. 

An  inability  to  fix  the  attention  is  a  remarkable  feature  of 
most  of  these  cases  during  examination. 

Cranial  nerves.  Primary  optic  atrophy,  similar  to  that 
found  in  tabes,  may  be  seen.  Oculo-motor  palsies  may 
also  occur,  but  are  probably  due  to  coexistent  intracranial 
syphilitic  lesions. 

Pupillary  changes  occur  early :  are  constant  and  charac- 
teristic, provided  the  significance  of  irregularity  in  outline, 
inequality  in  size  and  shape,  and  impaired  light-reaction  are 
fully  recognised.  In  many  cases  the  complete  Argyll-Robert- 
son reaction  is  present,  but  the  above-mentioned  changes  are 
equally  significant.  The  fifth  nerve  may  show  some  sensory 
anaesthesia,  especially  in  the  tabo-paretic  variety. 

The  other  motor    cranial  nerves    show  no   paralyses,   bu 
the    muscles    supplied   by   them   present   the   characteristic 
tremor  of  this  disease,  which  will  be  presently  described. 

The  motor  system.  A  characteristic  tremor  is  the  most 
constant  early  symptom  of  interference  with  the  motor 
functions.  It  is  seen  first  in  the  circum-oral  facial  muscles, 
lips,  and  tongue,  and  may  be  observed  at  rest ;  but  is 
accentuated  by  emotion,  attempts  to  speak,  and  on  protrusion 
of  the  tongue.  It  consists  of  irregular,  inconstant,  more  or 
less  rapid,  quivering  movements ;  rarely  confined  to  one 
muscle,  but  playing  over  groups  of  muscles.  The  same 
kind    of    tremor    is    seen   in    the    outstretched    hands   and 

26  * 


404  NERVOUS  DISEASES 

arms,  and  some  degree  of  incoordination  of  movement  is 
frequently  combined  with  it. 

The  articulation  is  slurring  and  tremulous :  the  commence- 
ment of  a  sentence  being  relatively  distinct,  but  terminating 
in  an  inarticulate  drawl.  Individual  syllables  may  be  repeated, 
or,  on  the  other  hand,  they  may  be  glossed  over.  The  most 
difficult  phrases  to  pronounce  are  those  in  which  the  tongue 
and  lips  are  specially  brought  into  action— for  example, 
'  Territorial  Artillery,'  and  '  West  Register  Street.'  In 
addition  to  the  articulatory  defect  the  patient  is  often  unable 
to  repeat  sentences,  owing  to  mental  impairment,  and  he 
may  also  be  temporarily  aphasic.  In  the  later  periods  he 
becomes  inarticulate. 

Voluntaiy  power  is  unaffected  in  the  early  stages ;  but,  as 
the  disease  progresses,  a  gradual  diminution  and  eventually 
complete  loss  of  motor  power  occurs.  The  finer  movements 
of  the  hands — as  in  writing — become  impaired  by  a  com- 
bination of  tremor  and  inco-ordination. 

The  muscular  tone  is  at  first  hypertonic ;  but  in  cases 
of  the  tabo-paretic  variety,  hypotonia  is  observed.  The  latter 
is  also  characterised  by  considerable  instability  and  ataxia. 

The  reflexes.  The  tendon  jerks  are  usually  exaggerated, 
but  may  be  lost  in  the  tabo-paretic  cases.  The  superficial 
reflexes  vary,  but  are  as  a  rule  present ;  the  plantars  are 
usually  flexor,  except  after  severe  epileptiform  seizures,  when 
they  show  the  extensor  response. 

The  sjjhincters,  especially  that  of  the  bladder,  are  affected 
early.  In  the  later  stages  of  dementia,  incontinence  and 
involuntary  evacuations  occur.  Sexual  power  is  often  lost 
in  the  early  stages,  but  in  other  cases  is  increased  almost  to 
the  extent  of  satyriasis.  Temporary  return  of  sexual  power 
has  been  known  to  occur  after  previous  abolition. 

Epileptiform  seizures  in  this  disease  are  of  three  kinds  : 
(a)  temporary  losses  of  consciousness,  followed  by  transient 
aphasia  ;  (b)  limited  Jacksonian  seizures,  followed  by  transient 
and  sometimes  permanent  loss  of  power ;  and  (c)  general 
epileptic  seizures.  During  the  severer  attacks  the  temperature 
is  increased  sometimes  to  hyperpyrexia. 

The  effect  of  such  attacks  is  to  aggravate  the  symptoms  of 
mental  decrepitude,  and  to  hasten  the  onward  progress  of  the 
disease. 


GENEKAL  DISEASES  OF  THE  NERVOUS  SYSTEM    405 

Sensory  symptoms.  Headache  and  cephalic  sensations  are 
frequent,  but  not  constant  symptoms  In  the  tabo-paretic 
cases  lightning  pains  and  the  objective  sensory  changes 
characteristic  of  tabes  are  present. 

Clinical  types.  (1)  Exalted  type.  This  is  a  form  pre- 
senting exaltation  and  grandiose  ideas,  with  incoherence  of 
speech.  In  many  respects  it  resembles  an  attack  of  acute 
maniacal  excitement. 

2.  A  melancholic  type,  rarer  than  the  preceding,  and 
presenting  a  very  definite  clinical  picture.  It  commonly 
commences  with  neurasthenic  symptoms,  which  precede  for 
a  longer  or  shorter  time  the  onset  of  obvious  delusional 
melancholia. 

3.  A  type,  which  may  be  called  the  tabo-paretic  type, 
where  the  disease  commences  with  the  symptoms  of  tabes 
dorsalis,  but  eventually  develops  mental  symptoms  of  general 
paralysis. 

4.  A  convulsive  type,  in  which  the  disease  commences 
with  a  series  of  one  or  more  general  epileptic  fits.  Upon 
recovery,  some  mental  or  motor  impairment  may  be  detected. 
The  patient  remains  in  this  state  until  again  prostrated  by 
a  further  series  of  convulsions. 

The  differential  diagnosis  has  to  be  made  in  the  early 
stage  from  neurasthenia  (p.  545),  and  in  the  later  from  diffuse 
cerebral  syphilitic  lesions  (p.  373;  and  tumour  of  the  frontal 
lobe  (p.  243).  In  all  doubtful  cases  of  neurasthenia  with 
a  history  of  syphilitic  infection,  a  cytological  examination 
of  the  cerebro-spinal  fluid  should  be  made.  An  excess  of 
lymphocytes  is  diagnostic  of  syphilitic  affections. 

From  diffuse  cerebral  syphilitic  disease  the  diagnosis  is 
difficult,  but  in  this  the  main  points  are :  the  occurrence 
of  paralyses  of  the  cranial  nerves  and  signs  of  gross  organic 
disease,  the  absence  of  grandiose  ideas,  and  of  the  charac- 
teristic tremors  and  slurring  speech  (p.  373). 

From  chronic  alcoholism,  a  knowledge  of  the  habits  of 
the  patient,  and  the  retention  of  the  pupillary  light- reflex, 
are  points  which  may  guide  the  observer. 

The  examination  of  the  cerebro-spinal  fluid  by  the  Wasser- 
man  reaction  is  more  likely  to  throw  light  upon  the  differential 
diagnosis  than  any  other  method.  This  is  a  complicated 
process,  but  in  the  hands  of  competent  persons  is  of  assured 


406  NKKVOl'S  DISEASES 

value.  Mott x  has  stated  that  a  positive  reaction  to  the 
WaBserman  test,  in  the  cerebro-spinal  fluid,  was  present  in 
89  per  cent,  of  his  cases ;  that  the  reaction  was  less  constant 
in  cases  of  tabes,  and  that  it  was  often  negative  in  syphilitic 
meningitis  and  diffuse  cerebral  syphilitic  lesions.  In  all  the 
reaction  of  the  blood  serum,  may  be  positive.  The  reaction 
is  probably  not  present  in  any  other  diseases. 

Prognosis.  The  average  duration  of  the  disease  is  about 
three  }Tears  ;  rarely  do  these  patients  live  more  than  five. 
Many  general  paralytics  however  die,  in  the  ordinary  course 
of  their  malady,  well  under  three  years.  Their  tenure  of  life  is, 
moreover,  very  uncertain :  as  death  may  result  in  consequence 
of  an  epileptiform  seizure,  and  there  is  great  liability  to  inter- 
current complications.  On  the  other  hand,  there  are  cases 
which  run  a  rapid  course  of  a  few  months,  and  die  either 
from  the  intensity  of  the  mental  symptoms,  or  from  inter- 
current pulmonary  complications.  In  the  ordinary  course 
death  takes  place  from  inanition,  bedsores,  cystitis,  pulmonary 
troubles,  and  sometimes  from  status  epilepticus. 

Treatment.  In  the  early  stages  an  energetic  course  of 
antisyphilitic  treatment,  followed  by  general  tonic  remedies, 
is  important  in  cases  which  have  not  been  previously  treated 
for  syphilis. 

The  patient  should  be  removed  from  work  and  advised  to 
live  a  quiet  life  in  the  country.  He  should  be  protected  from 
the  consequences  of  ill-balanced  action  or  erratic  procedures. 

In  many  cases  it  is  necessary  to  certify  and  send  him  to 
an  asylum  ;  or,  at  all  events,  to  have  him  under  care  and 
supervision  in  a  sanatorium. 

Juvenile  general  paralysis 

This  condition  is  intimately  associated  with  inherited 
syphilis,  and  occurs  with  equal  frequency  in  both  sexes.  It 
commences  between  the  ages  of  eight  and  twenty  years,  but 
not  infrequently  engrafts  itself  upon  an  already  defective 
nervous  system. 

The  morbid  anatomy  is  similar  to  that  described  in  the 
adult  form,  with,  in  addition,  signs  of  congenital  syphilis  In 
other  parts  of  the  body. 

1  Mott,  Lancet,  1909. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    407 

As  a  rule  the  symptoms  develop  gradually.  The  mental 
changes  commence  with  depression,  exaltation,  or  perversion, 
and  tend  to  progress  to  a  state  of  complete  dementia. 
Grandiose  ideas,  delusions,  and  outbursts  of  acute  excitement 
may  occur,  but  are  less  common  than  in  the  adult  form.  On 
examination  the  patients  invariably  present  the  features  of 
congenital  syphilis.  They  are  small  and  poorly  developed, 
with  absence  of  pubic  hair,  and  ill-defined  breasts  and 
testicles ;  scarring  about  the  angles  of  the  mouth,  peg-shaped 
teeth,  interstitial  keratitis,  and  choroidal  atrophy.  The 
pupillary  light-reaction  is  absent,  and  the  deep  reflexes  are 
in  some  brisk ;  in  others,  abolished.  They  present  the 
characteristic  tremor  of  the  face,  tongue,  and  limbs  already 
described,  and  the  articulation  is  slurring.  As  in  juvenile 
tabes,  optic  atrophy  is  a  common  and  sometimes  an  early 
sign. 

The  ijrognosis  is  bad,  as  the  disease  usually  terminates 
fatally  in  two  or  three  years ;  but  in  some  cases  life  may  be 
prolonged  for  five  or  six  years.  There  is  progressive  mental 
and  physical  weakness,  which  renders  the  patient  liable  to 
any  intercurrent  affection. 


Chaptee  III 

CHRONIC    ATROPHIC    PARALYSES     OF    SPINAL 
AND    BULBAR    ORIGIN 

Under  this  title  are  grouped  and  described  three  closely 
allied  disorders — progressive  muscular  atrophy,  amyotrophic 
lateral  sclerosis,  and  bulbar  paralysis.  The  difference  in 
their  clinical  features  depends  chiefly  upon  the  seat  of  the 
lesion. 

1.  Progressive  muscular  atrophy  is  a  malady  characterised* 
by  progressive  wasting  of  certain  muscles,  or  groups  of  muscles, 
unaccompanied  by  sensory  changes,  and  without  obvious- 
evidence  of  lesion  of  the  upper  motor  neurone.  It  is  probable, 
however,  that  few  pure  cases  of  this  type  exist,  as  almost  in- 
variably some  coexistent  alterations  in  the  reflexes,  and  slight 
spasticity,  may  be  found  associated  pathologically  with  early 
degenerative  changes  in  the  pyramidal  system. 


408 


NERVOUS  DISEASES 


•2.  Amyotrophic  lateral  sclerosis.  The  symptoms  consist 
primarily  of  muscular  wasting— commonly  of  the  distal  por- 
tions of  the  upper  limbs — without  sensory  impairment,  and 
progressive  spastic  paraplegia,  in  which  the  upper  limbs  also 
participate.     They  are  due  to  degenerative  atrophy  of  the 


Fig.  122. 


Fig.  1  23. 


Fig.  124. 


Figs.  122 -124. — These  figures  illustrate  the  pathological  changes  in  the  spinal 
cord  from  a  case  of  amyotrophic  lateral  sclerosis.  The  posterior  columns 
are  normal ;  an  extensive  degeneration  is  seen  in  the  antero-lateral 
columns,  and  especially  in  the  crossed  pyramidal  tracts.  The  anterior 
horns  are  wasted. 


ganglion  cells  of  the  anterior  horns,  along  with  well-marked 
sclerotic  changes  of  the  pyramidal  fibres.  Most  of  the  cases 
of  amyotrophic  lateral  sclerosis  develop  bulbar  symptoms,  as 
the  result  of  similar  atrophic  changes  in  the  ganglion  cells 
of  the  bulbar  nuclei. 

3.  Bulbar  paralysis  is  characterised  by  muscular  wasting, 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    409 

commencing  primarily  in  the  muscles  supplied  from  the 
bulbar  motor  nuclei.  In  this  type  the  atrophic  changes  go 
on  to  affect  the  anterior  cornual  regions  of  the  spinal  cord, 
and  the  pyramidal  implication  is  early  and  well  marked. 

A  family  form  occurring  in  children  ( Werdnig-Hoffmann; 
is  described  on  p.  471. 

PROGRESSIVE   MUSCULAR   ATROPHY 

Pure  cases  of  this  form  of  chronic  atrophic  paralysis  are 
rare.  Males  are  affected  more  frequently  than  females,  and 
in  both  sexes  the  age  at  onset  is  usually  after  thirty. 


Fig.  125. — Showing  atrophy  of  the  small  muscles  of  the  hands 
in  an  early  case  of  progressive  muscular  atrophy. 

Symptoms.  The  onset  of  the  disease  is  insidious :  the 
patient's  attention  being  first  drawn  to  the  condition  by  the 
atrophy  of  the  small  muscles  of  the  hands.  In  the  early 
stages  the  patient  notices  that  his  hands  are  getting  thinner 
before  he  is  conscious  of  any  loss  of  power.  As  the  atrophy 
progresses,  weakness  of  the  hands  develops — especially  for 
holding  objects  with  the  thumb  and  fingers.  The  thenar 
and  hypothenar  eminences  waste,  and  the  metacarpal  bones 
stand  out  prominently  owing  to  the  atrophy  of  the  lumbricales 
and  interossei.  Fibrillary  tremor  may  be  observed  by  the 
patient,  or  may  give  rise  to  a  sensation  of  '  fluttering  under 
the  skin.'  As  time  goes  on  the  muscles  of  the  forearm  are 
involved,  and  later  those  of  the  shoulder,  so  that  the  patient 
is  unable  to  raise  his  arms  above  his  head.  If  the  disease 
progresses,  the  neck,  muscles,  and  those  of  the  lower  limbs, 


410 


NERVOUS  DISEASES 


may  atrophy  and  the  patient  become  bedridden  in  from  nine 
(o  eighteen  months.  In  pure  cases,  however,  the  extension  of 
the  disease  is  slow,  and  the  atrophy  may  remain  limited  to 
the  small  muscles  of  the  hand  alone.  In  cases  where  the 
atrophy  is  extensive,  complaint  may  be  made  of  dull  aching 
pain  in  the  limbs,  due  to  the  extra  strain  on  the  weakened 
muscles.  This  pain  is  not  attended  by  any  muscular  hyper- 
esthesia nor    by  objective  sensory  changes.     Some    idea  of 

the  rate  of  progress  may  be 
obtained  by  observing  the  distri- 
bution of  the  fibrillary  tremor 
which,  as  a  rule,  precedes  the 
onset  of  the  atrophy  ;  thus  in 
any  case  where  such  tremor 
is  widespread  the  prognosis  is 
most  unfavourable. 

The  mental  condition  is  unim- 
paired. 

The  cranial  nerves.  These 
are  not  affected  except  in  the 
advanced  cases  when  the  bulbar 
nuclei  have  become  involved, 
and  atrophy  of  the  muscles 
supplied  by  the  spinal  accessory 
nerve  —  trapezii  and  sterno- 
mastoids  —  and  later  of  the 
tongue  and  palatal  muscles 
ensues. 

The  motor  system.  The 
muscles  most  commonly  affected 
are  the  small  muscles  of  the  hand,  the  muscles  of  the 
shoulder  and  upper  extremities,  neck,  lower  limbs,  and 
trunk.  Fibrillary  tremor  is  a  constant  and  characteristic 
feature,  and  the  electrical  reactions  show  all  stages  of  the 
reaction  of  degeneration,  from  mere  diminution  of  faradic 
excitability  to  the  fully  developed  reaction  of  degeneration. 
On  striking  the  muscle  a  slow  and  prolonged  contraction 
takes  place.  It  is  characteristic  of  the  malady  that  in  the 
early  stages  the  muscular  atrophy  is  out  of  all  proportion  to 
the  loss  of  power,  which  is  not  complained  of  until  the 
wasting  of  the  muscles  is  well  advanced.     The  loss  of  power 


Fig.  126. — Illustrates  the  appear- 
ance of  the  shoulders  and  arms 
in  a  case  of  progressive  muscu- 
lar atrophy. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    411 

is  always  less  than  the  appearance  of  the  affected  muscles 
would  suggest. 

Sensory  system.  Subjective  sensations  of  aching  in  the 
limbs,  and  of  '  fluttering '  under  the  skin,  are  sometimes 
complained  of ;  but  these  sensations  are  due  to  the  weakness 
of  the  muscles  and  the  presence  of  fibrillary  tremor.  There 
is  no  objective  loss  of  sensibility. 

Reflexes.  The  deep  reflexes  disappear  concurrently  with 
the  muscular  atrophy,  but  may  remain  unaffected  in  quality, 
though  reduced  in  quantity,  as  long  as  normal  muscle 
remains.  Where  the  muscles  are  not  wasted  the  deep  reflexes 
remain  normal  or  are  slightly  increased.  Exaggeration  of 
the  deep  reflexes  and  clonus  only  occur  when  the  pyramidal 
tracts  are  involved — that  is,  in  cases  of  amyotrophic  lateral 
sclerosis. 

The  superficial  reflexes  remain  normal  in  all  pure  cases 
of  progressive  muscular  atrophy ;  but  where  pyramidal 
affection  develops,  the  abdominal  and  epigastric  reflexes 
become  diminished,  and  the  plantar  reflexes  become  extensor 
in  type. 

Sphincters.  There  is  no  affection  of  the  sphincters  in  the 
pure  cases. 

Clinical  types,  (1)  The  common  type.  The  wasting 
commences  in  the  small  muscles  of  the  hands,  and  may 
either  extend  to  and  involve  other  muscles,  or  remain  more 
or  less  limited  to  the  small  muscles. 

(2)  A  type  in  which  the  atrophy  first  affects  the  muscles 
of  the  shoulder  girdle  and  upper  arm.  This  is  comparatively 
rare,  and  is  seen  generally  in  persons  who  have  been  exposed 
to  some  chronic  intoxication — such  as  lead.     (Fig.  126.) 

(3)  A  type  in  which  the  muscles  of  the  lower  extremities 
are  first  affected. 

Differential  diagnosis.  Progressive  muscular  atrophy  has- 
to  be  distinguished  from — 

1.  The  muscular  dystrophies  (p.  45;  by  : 

(a)  The  absence  of  any  family  history. 

(b)  The  age  at  onset. 

(c)  The    distribution    of    the    atrophy   which    is    not 

that     of     any     of    the    characteristic    types    of 
myopathy. 

(d)  The  presence  of  fibrillary  tremor. 


412  NERVOUS  DISEASES 

(e)  The   presence   of  the  reaction  of  degeneration  on 
electrical  examination. 

2.  Peroneal  muscular  atrophy  (p.  472)  by: 

{a)  The  absence  of  any  family  history  of  the  disease. 

(b)  The  age  at  onset. 

(c)  The  distribution  of  the   atrophy  which    tends   to 

involve    groups    of    muscles    other    than    those 
affected  in  peroneal  muscular  atrophy. 

{<!)  The  absence  of  sensory  changes. 

(e)  The  progress  and  extension  of  the  disease. 

3.  The     muscular    wasting    associated    with    cervical    ribs 
(p.   110)  by  : 

(a)  The  bilateral  distribution  of  the  atrophy. 

(b)  The  absence  of  sensory  changes. 

(c)  The  progressive  extension  of  the  atrophy. 

(d)  The  negative  result  of  an  X-ray  examination. 

4.  Lesions  of  the  peripheral  nerves  (p.  98)  by: 

(a)  The  absence  of  a  local  or  general  cause. 
(&)  The  absence  of  pain  or  sensory  loss. 

(c)  The    distribution   of  the   paralysis,  which    is   not 

according  to  root  or  nerve  lesion. 

(d)  The  absence  of  trophic  changes  other  than  muscular. 

4.  Arthritic  muscular  atrophy  (p.  99)  by  : 

(a)  The  absence  of  arthritic  changes. 

(6)  The  distribution  of  the  atrophy  which  is  not  related 

to  an}r  joint,  and  the  presence  of  R.D. 
(o)  The  evidence  of  intramedullary  changes. 

5.  Craft  palsies  (p.  513)  by  : 

(a)  The  absence  of  a  history  of  employment  in  an  occu- 
pation likely  to  cause  a  toxic  or  pressure  paralysis. 
(6)  The  absence  of  paresthesia. 

{<■)  The  absence  of  evidence  of  lesion  of  a  peripheral 
nerve. 
Prognosis.  This  depends  on  the  rate  of  development  of 
the  atrophy.  If  after  six  months  the  atrophy  is  still  limited 
to  the  muscles  originally  affected,  the  progress  of  the  disease 
is  likely  to  be  slow,  although  no  recovery  of  the  wasted 
muscles  will  occur. 

The  prognosis  is  bad  if  there  be  along  with  the  muscular 
atrophy  much  fibrillation  in  other  muscles. 

In  the    rapid  cases  death   usually   occurs  in  from   twelve 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    413 

months  to  two  years ;  but  in  the  more  chronic  cases  life  may 
be  prolonged  for  five  or  fifteen  years,  provided  that  bulbar 
symptoms  do  not  supervene  and  that  serious  pulmonary 
complications  are  avoided. 

Treatment.  The  most  valuable  medicinal  remedy  is  the 
hypodermic  injection  of  strychnine  (-g-1^  grain  to  TV  grain). 
This  should  be  supplemented  by  general  tonic  treatment, 
fresh  air,  and  the  avoidance  of  fatigue — more  especially  of  the 


Fig.  127. — Illustrates  the  appearance   of  the  hands   in   an  early 
stage  of  amyotrophic  lateral  sclerosis. 

affected  limbs.  Treatment  should  also  be  directed  to  any 
conditions  which  may  be  possible  causal  factors.  The  local 
treatment  consists  of  galvano-faradism  and  massage  of  the 
atrophied  muscles.  This  ought  to  be  applied  short  of  fatigue. 
Although  most  cases  are  progressive,  yet  the  employment  of 
treatment,  as  above  described,  may  lead  to  an  arrest  of  the 
disease. 


AMYOTROPHIC   LATERAL    SCLEROSIS 

In  this  condition  there  is,  in  addition  to  the  degeneration 
of  the  anterior  horn  cells,  a  sclerosis  of  the  pyramidal  system 
extending  up  to  the  cortex  cerebri. 

Symptoms.     The  symptoms  vary  at  the  outset  according 


414  XERYOUS  DISEASES 

to  whether  the  anterior  horns  or  the  pyramidal  system  are 
first  affected.  Generally  both  are  involved  simultaneously, 
so  that  a  condition  of  atrophic  paralysis  in  the  small  muscles 
of  the  hands  is  present  coincidently  with  a  spastic  paresis  of 
the  lower  limbs.  As  the  disease  progresses,  and  it  is 
essentially  a  progressive  disorder,  the  spasticity  of  the  lower 
limbs    increases   and   the   wasting  extends  to  other  muscles 


Fig.  128. — Illustrates  the  appearance  of  the  hands  in  an  advanced 
case  of  amyotrophic  lateral  sclerosis. 

of  the  upper  extremities.  At  this  stage  the  arms  are  wasted 
and  useless,  and  walking  is  difficult  owing  to  the  weakness 
and  spasticity  of  the  legs.  Still  later,  bulbar  symptoms 
arise  and  the  patient  has  difficulty  in  swallowing  and  in 
articulation,  and  the  muscles  of  the  lower  limbs  commence 
to  atrophy,  so  that  the  spastic  paralysis  gives  place  to 
atrophic  flaccid  paralysis. 

The  condition  of  the  atrophied  muscles  is  similar  to  that 
seen  in  progressive  muscular  atrophy ;  but  weakness  and 
spasticity  are  found  in  those  which  are  not  atrophied. 

The  atrophy  involves  specially  the  small  muscles  of  the 
hand  and  those  of  the  arm,  shoulder,  and  neck  ;  then  the 
bulbar  muscles  and  those  of  the  lower  limbs. 

Sensory  system.  There  is  no  disturbance  of  the  sensory 
functions  beyond  painful  reflex  spasms  in  the  legs,  aching 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM     415 

pain  in  the  upper  limbs  due  to  the  muscular  atrophy,  and 
the  sensation  due  to  the  fibrillary  tremor. 

Beflexes.  The  deep  reflexes  are  increased,  and  clonus 
always  develops  in  the  later  stages. 

The  superficial,  epigastric,  and  abdominal  reflexes  are 
abolished,  and  the  plantar  reflexes  are  extensor  in  type. 

The  sphincters  are  slightly  affected ;  some  delay  in 
micturition  may  take  place,  especially  when  the  patient  is 


Fig.  129. 


Fig.  130. 


Figs.   129   and   130. — Two   photographs   showing  the    facial   appearance  on 
laughing,  and  on  attempts  to  protrude  the  tongue,  in  bulbar  paralysis. 


bedridden  ;  but  there  is  never  incontinence  of  urine.  Con- 
stipation may  become  obstinate. 

Clinical  types.  In  some  cases  the  development  of  the 
disease  is  slow,  and  the  patient  may  live  from  three  to  five 
years.  It  may  commence  with  atrophy  of  the  small  muscles 
of  the  hand  or  with  spastic  weakness  of  the  legs,  but  even- 
tually both  these  conditions  are  found  in  association  with 
each  other. 

In  other  cases  the  progress  of  the  disease  is  rapid  with 
widespread  muscular  wasting  and  bulbar  paralysis.  The 
cases  in  which  the  bulbar  nuclei  are  involved  end  fatally  in 
from  ten  to  eighteen  months. 


416 


NERVOUS  DISEASES 


Bulbab   Paralysis   \m>  Chronic  Ophthalmoplegia 

The  pathological  basis  of  both  these  conditions  is  similar 
to  that  of  progressive  muscular  atrophy  and  amyotrophic 
lateral  sclerosis,  and  differs  only  in  the  early  implication  of 
the  cranial  nerve  nuclei. 

In  the  bulbar  cases  it  is  rare  for  the  disease  to  remain 
limited    to    the    bulbar    nuclei,    as    it   tends    to    involve    the 


Fie.  131. — Tlic  face  in  a  case  of  complete  nuclear  ophthalmo- 
plegia. Observe  especially  the  partial  double  ptosis,  the 
overaction  of  the  frontalis,  and  tilting  back  of  the  head. 


pyramidal  system  and  the  anterior  horn  cells  in  the  spinal 
cord. 

Symptoms.  These  are  wasting  and  degeneration  of  the 
muscles  supplied  from  the  bulbar  nuclei,  wasting  and 
atrophy  of  the  tongue,  which  cannot  be  protruded,  atrophy 
of  the  palatal  and  pharyngeal  muscles,  and  often  of  the 
upper  part  of  the  trapezius  and  sterno-mastoid  muscles, 
and  sometimes  of  the  face. 

In   some  cases  the  disease  affects  the  upper  cranial  neive 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    417 

nuclei,  giving  rise  to  a  chronic  ophthalmoplegia,  often  limited 
to  the  external  muscles  of  the  eyes,  but  sometimes  affecting 
the  pupillary  muscles  as  well.  The  paralysis  may  remain 
limited  to  the  ocular  muscles,  or  may  extend  to  the  bulbar 
and  spinal  muscles.  In  rare  cases  the  ocular  muscles  are 
involved  secondarily  to  bulbar  or  spinal  paralysis. 

Differential  diagnosis.     Amyotrophic  lateral  sclerosis  and 
bulbar  paralysis  have  to  be  distinguished  from — 

1.  Intramedullary  lesions  (tumour,  syringomyelia)  (p.  328), 
by: 

(a)  The  absence  of  pain. 

(b)  The  bilateral  and  symmetrical  progress  and  dis- 

tribution of  the  atrophy. 

(c)  The  absence  of  sensory  loss. 

(d)  The  absence  of  trophic  changes  other  than  muscular. 

(e)  The  non-involvement  of  the  sphincters. 

2.  Extramedullary    lesions     affecting    the    cord    or    spinal 
roots  (p.  350),  by  : 

(a)  The  absence  of  root  pains. 

(b)  The  distribution  of  the  paralysis  which  is  not  that 

of  root  paralysis. 

(c)  The  absence  of  sensory  loss. 

(d)  The  absence  of  a  definite  segmental  level  of  para- 

lysis, sensory  or  motor. 

(e)  The  course  and  spread  of  the  disease. 
(/)  The  non-involvement  of  the  sphincters. 

Bulbar   palsy   and   chronic    ophthalmoplegia   have   to    be 
distinguished  from — 

1.  Myasthenia  gravis  (p.  485),  by  : 

(a)  The  absence  of  remission  and  relapse  of  symptoms. 

(b)  The  presence  of  atrophy  with  relatively  slight  loss 

of  motor  power,  whereas  in  myasthenia  the  loss 
of  power  is  great  with  little  or  no  atrophy. 

(c)  The  absence  of  the  myasthenic  reaction  and  the 

presence  of  the  reaction  of  degeneration. 

(d)  The  presence  of  signs  of  lesion  of  the  pyramidal 

tracts. 

2.  Double  hemiplegia  (p.  188),  by: 

(a)  The  absence  of  a  history  of  a  stroke  or  strokes. 

(b)  The   presence   of    atrophy    with   the    reaction    of 

degeneration. 

27 


418  XHK  Vol's  D1SKASKS 

The  prognosis  in  cases  of  bulbar  paralysis  is  unfavour- 
able, some  cases  ending  fatally  in  six  months  from  failure  of 
the  respiratory  and  cardiac  centres.  Few  cases  survive  more 
than  three  years.  Where  the  paralysis  is  limited  to  the 
ocular  muscles,  the  prognosis  as  regards  life  is  not  so  un- 
favourable, many  cases  living  for  ten  to  fifteen  years  after 
the  onset  of  the  symptoms. 

Treatment.  This  is  similar  to  that  described  under  pro- 
gressive muscular  atrophy  ;  but  in  addition  means  ought  to  be 
taken  to  allay  spasticity  and  to  prevent  contractures.  These 
objects  are  best  attained  by  the  employment  of  local  hot-air 
baths,  followed  by  massage  and  passive  movements.  Walk- 
ing should  be  permitted  for  periods  well  within  the  limits  of 
fatigue.    The  chances  of  arrest  of  the  disease  are  less  hopeful. 

In  the  bulbar  forms  special  attention  has  to  be  given  to 
feeding,  semi-solid  food  being  swallowed  more  easily  than 
liquid  or  solid  food.  Care  should  be  taken  in  the  later  stages 
to  prevent  the  passage  of  food  into  the  larynx,  nasal  feed- 
ing sometimes  being  necessary.  Pulmonary  and  cardiac 
complications  need  careful  attention. 


Chapter    IV 

DISSEMINATED    SCLEROSIS 

(syn.:    sclerose    en  plaques multiple   cerebrospinal 

sclerosis) 

This  is  a  chronic  progressive  disease,  characterised  patho- 
logically by  the  presence  of  patches  of  sclerosis  situated  at 
random  throughout  the  central  nervous  system,  and  clinically 
by  paresthesia,  spastic  paralysis,  interference  with  the 
action  of  the  sphincters,  tremors  on  volitional  effort,  articu- 
latory  disability,  and  sometimes  by  optic  atrophy  and  other 
cranial  nerve  palsies. 

Etiology.  This  disease  commonly  commences  in  early 
adult  life,  but  may  arise  at  any  age  from  twenty  to  forty-five. 
Its  occurrence  in  children  has  been  described,  but  it  is  doubtful 
whether  these  have  been  cases  of  the  classical  disease  as  seen 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    419 

in  young  adults.     Out  of  three  hundred  cases  which  we  have 
personally  observed  there  were  only  two  in  which  the  symptoms 


^ 

|l|fllfl| 

m£r 

;  < 

r0* 

"j*$ 

. '^gSS 

mm 

"^Sks^ 

n 

>  ■  'iai 

iflAEi^ 

-""':::y 

-:;','        :'       • 

Figs.  132  and  133  are  two  sections  of  the  spinal  cord  from 
a  case  of  disseminated  sclerosis.  In  Fig.  132  the  patch  of 
sclerosis  occupies  the  posterior  columns ;  in  Fig.  133  the 
patch  is  in  the  antero-lateral  portion  of  the  cord  on  the 
right-hand  side,  involving  both  white  and  grev  matter. 


commenced  before  the  sixteenth  year,  one  case  at  fourteen 
and  one  at  fifteen.     Its  onset  after  fifty  is  rare. 

It  occurs  rather  more  frequently  in  males  than  in  females. 


27  * 


■420  NERVOUS  DISEASES 

There  is  little  that  is  certain  known  about  its  causation. 
It  has  no  relation  to  syphilis. 

Certain  conditions,  however,  such  as  pregnancy,  fatigue, 
and  acute  pyrexial  disorders,  have  a  deleterious  influence 
upon  the  developed  malady. 

Pathological  anatomy.  On  palpation  of  the  cord,  the 
surface  presents  irregular  hard  lumps  which  may  be  felt 
by  the  finger.  To  the  naked  eye,  when  sections  are  made 
through  the  brain  or  spinal  cord,  irregularly  shaped  and 
diffusely  scattered  patches  of  various  sizes  of  a  pinkish  and 
semi-translucent  appearance  are  seen.  These  are  well  defined, 
present  a  sharp  edge,  and  stand  out  clearly  from  the  adjacent 
nerve  tissue.  They  are  most  common  in  the  spinal  cord, 
the  mid  and  hind  brain,  and  the  centrum  ovale,  but  may  be 
found  in  any  part  of  the  central  nervous  system.  They  affect 
indiscriminately  both  the  grey  and  the  white  matter.  Similar 
patches  have  been  observed  in  the  optic  and  spinal  nerves. 

The  chief  changes  noticed  on  microscopical  examination  of 
a  sclerotic  patch  are  :  the  absence  of  the  myeline  sheaths 
of  the  nerves,  the  retention  of  normal  or  perhaps  somewhat 
atrophied  axis-cylinder  processes,  and  the  presence  of  a  dense 
network  of  neuroglial  fibrils  with  spider-cells  and  nuclear 
infiltration.  The  retention  of  the  axis-cylinder  processes  and 
their  normal  reaction  to  the  usual  staining  reagents  are  the 
characteristic  pathological  features  of  the  disease.  There  may 
be  no  signs  of  Wallerian  degeneration  of  the  tracts  either  above 
or  below  the  sclerotic  areas,  a  fact  which  is  probably  explained 
by  the  retention  of  the  axis- cylinders  of  the  nerve  fibres. 
Should  a  patch  of  sclerosis  implicate  the  grey  matter,  the 
ganglion  cells  may  undergo  some  degenerative  alteration,  but 
such  changes  are  of  slow  development. 

In  the  advanced  stages  of  the  disease  large  areas  of 
sclerosis  are  observed,  in  some  places  throughout  the  whole 
cross- section  of  the  cord ;  in  other  places  small  islets  only 
of  apparently  normal  nerve  tissue  may  be  detected  within 
the  areas  of  sclerosis.  In  the  vast  majority  of  cases  the 
transition  between  the  normal  tissue  and  the  sclerosed  area  is 
quite  abrupt.  Vascular  changes  are  not  characteristic,  but 
sometimes  hyaline  degeneration  of  the  vessel  walls  is  present. 
At  the  borders  of  recent  patches  compound  granular  cells  are 
sometimes  found.     (Figs.  132  and  133.) 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    421 

No  satisfactory  explanation  to  account  for  the  formation  of 
the  disseminated  patches  has  yet  been  given.  Three  theories 
have  been  advanced  :  first,  that  the  condition  is  due  to  a 
congenital  defect  in  the  development  of  the  myeline  sheaths  in 
certain  localities ;  secondly,  that  the  sclerosis  is  primarily  of 
vascular  origin ;  and,  thirdly,  that  the  disease  is  an  inflam- 
mation of  the  neuroglial  tissues,  which  destroys  the  myeline 
sheaths  around  the  axis-cylinders. 

Symptoms.  The  symptoms  necessarily  depend,  in  part 
upon  the  situation  of  the  lesions,  and  in  part  upon  the  number 
of  the  islets  of  sclerosis.  In  some  cases  the  spinal  cord  may 
be  the  primary  seat  of  the  disease,  when  the  early  symptoms 
are  more  or  less  paraplegic  in  type  ;  in  others  the  lesions  may 
primarily  be  limited  to  the  brain,  with  monoplegic  or  hemi- 
plegic  symptoms ;  in  others  again  the  cranial  nerves  may  be 
affected,  the  early  symptoms  being  diplopia  from  oculo-motor 
paralysis  or  loss  of  sight  from  optic  nerve  atrophy.  It  is 
obvious,  therefore,  that  great  variability  and  irregularity  may 
characterise  the  onset  of  this  disease  ;  but  in  the  later  and 
terminal  stages  the  clinical  picture  is  much  the  same,  in  what- 
ever way  the  disease  commences. 

A  tendency  to  remission  and  relapse  of  symptoms  is  a 
highly  characteristic  feature  of  this  malady,  the  relapse  not 
necessarily  occurring  in  the  same  part  of  the  body  as  that 
originally  affected.  Thus  the  first  symptom  may  be  an  oculo- 
motor palsy,  which  disappears,  and  is  followed  later  on  by 
paraplegia  or  sphincter  trouble. 

The  mental  condition.  Most  cases,  especially  those 
originating  in  early  adult  life,  exhibit  a  peculiar  and  more  or 
less  characteristic  mental  attitude.  There  is  some  mental 
dullness,  the  temperament  is  peculiarly  cheerful,  combined 
with  a  tendency  to  slight  childishness,  and  an  abnormal 
optimism  as  to  the  progress  of  the  malady.  The  facial 
expression  is  vacant  or  blank,  but  may  be  broken  in  upon 
by  smiles  and  laughter  aroused  hj  trivial  circumstances. 

Special  senses.  Smell,  taste,  and  hearing  are  rarely 
affected.  Impairment  of  vision  (amblyopia)  may  be  an  early 
symptom.  The  following  varieties  are  found :  (1)  amblyopia 
over  the  whole  field  with  contraction  of  the  visual  area ; 
(2)  central  scotoma.  Both  of  these  conditions  may,  or  may 
not,  be  associated  with  optic  atrophy,  and  may  be  confined  to 


422  NERVOUS  DISEASES 

one  eye.  The  onset  of  these  symptoms  may  be  sudden  or 
gradual..  In  cases  where  the  onset  is  sudden,  remissions 
frequently  take  place.  (3)  Hemianopsia  and  quadrantic  loss 
of  vision  may  be  observed  in  other  cases,  as  a  result  of 
sclerotic  patches  in  the  primary  or  higher  visual  tracts. 
Vision  is  rarely  completely  lost. 

Optic  atrophy.  Pallor  of  one  or  both  optic  discs  is  present 
in  about  50  per  cent,  of  the  cases  when  the  disease  is  fully 
developed.1  The  colour  of  the  disc  is  usually  a  papery  white, 
the  pallor  being  more  pronounced  upon  the  temporal  side  of 
the  disc.  The  atrophied  disc  never  shows  the  grey  tint  which 
is  so  characteristic  of  tabetic  atrophy,  and  the  blood-vessels 
of  the  fundus  stand  out  clearly,  and  rarely  present  signs  of 
inliammatory  or  vascular  lesion. 

Papillitis.  In  some  of  the  more  acute  cases,  especially 
those  with  sudden  onset  of  impairment  of  vision,  a  mild 
degree  of  papillitis  may  be  observed  suggestive  of  a  retrobul- 
bar neuritis.  There  is  no  evidence  that  it  is  an  inflammatory 
lesion.  It  is  stated  by  Uhthoff  to  occur  in  5  per  cent,  of  the 
cases.  The  condition  is  transitory  and  gives  place  to  optic 
atrophy,  with  temporary  or  permanent  loss  of  central  vision. 

Oculo-motor  nerves.  The  pupils  are  either  of  medium  size  or 
large.  Their  reaction  to  light  is  not  impaired,  except  in  those 
cases  in  which  optic  atrophy  is  present. 

Diplopia,  most  commonly  resulting  from  paralysis  of  the 
sixth  nerve,  is  sometimes  seen,  and  is  often  an  early  and 
transient  symptom.  Inquiry  should  always  be  directed  as 
to  antecedent  diplopia.  Occasionally  the  third  and  fourth 
nerves  may  be  affected. 

Nystagmus,  especially  on  lateral  movement,  is  a  very 
characteristic  sign.  Although  commonly  bilateral,  it  may  be 
present  on  one  side  only.  The  movements  may  be  fine  or 
coarse,  and  are  often  quite  irregular.  In  some  cases  nystagmus 
is  absent,  but  in  other  cases  it  is  spontaneous,  especially  when 
there  is  optic  atrophy. 

Affections  of  the  other  cranial  nerves  are  rare,  but  spastic 
weakness  and  inco-ordination  of  the  muscles  supplied  by  the 
seventh,  ninth,  tenth,  and  twelfth  nerves  give  rise  to  defects 
of  articulation,  and  are  not  uncommon  in  the  fully  developed 

1  Buzzard  (Thomas),  Simulation  of  Hysteria  by  Organic  Disease,  1891. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    423 

disease.  '  Nystagmus  '  of  the  palate  and  of  the  vocal  cords 
has  been  observed. 

Speech  defects  are  entirely  articulatory,  and  give  rise  to 
the  '  staccato,'  scanning  or  syllabic  utterance,  which,  though 
a  classical  sign,  is  of  rare  occurrence.  Minor  degrees  of 
articulatory  impediment  are,  however,  not  uncommon, 
especially  indistinct  pronunciation  of  the  explosives,  b,  d,  g, 
and  a  monotonous  voice,  in  which  the  syllables  are  uttered 
hesitatingly  and  slowly. 

Motor  system.  The  motor  affections  vary  in  degree  and 
extent,  are  often  transitory,  but  tend  to  progress.  The 
paresis,  or  paralysis,  is  as  a  rule  one  of  movements  rather 
than  of  muscles,  and  of  the  upper  neurone  type,  namely 
spasticity  without  muscular  atrophy  or  changes  in  the 
electrical  reactions. 

In  the  early  stages  and  in  slight  cases  spasticity  may  be 
almost  negligible ;  but  in  the  severe  cases  may  be  profound, 
and  lead  to  flexor  and  adductor  contractures  of  the  lower 
limbs.  Another  feature  of  the  motor  affection  is  the  readiness 
with  which  fatigue  sets  in. 

Muscular  wasting  from  affection  of  the  anterior  cornual 
region  occurs  in  a  very  small  percentage  of  cases. 

The  characteristic  '  intention  '  or  '  action  '  tremor,  which  is 
one  of  the  classical  signs  of  the  malady,  is  almost  invariably 
present  in  greater  or  less  degree ;  and  as  a  rule  the  resulting 
unsteadiness  is  the  chief  disablement  of  the  upper  limbs.  It 
is  static  in  character,  and  may  also  be  seen  in  the  head  and 
trunk  as  well  as  in  the  limbs.  It  may,  indeed,  be  present 
under  all  circumstances,  except  that  of  complete  rest  in  bed. 
In  its  minor  degrees  it  may  be  demonstrated  by  asking  the 
patient  to  approximate  the  points  of  his  fingers  or  to  touch 
the  point  of  his  nose  ;  and  in  contradistinction  to  cerebellar 
ataxy,  the  unsteadiness  and  movements  are  most  marked 
when  the  ultimate  object  is  achieved.  The  cause  of  this 
tremor  is  quite  obscure,  but  there  is  no  evidence  to  support 
the  view  that  the  motor  impulses,  which  are  directed  along 
fibres  whose  axis  -  cylinders  are  denuded  of  their  myeline 
sheaths,  are  retarded  in  their  passage  through  the  cord.  It 
is  more  likely  that  it  is  due  to  interference  with  the 
efferent  cerebellar  tracts  and  their  connexions. 

The  gait   may  be    spastic-paretic   in   character,    the   toes 


424  NERVOUS  DISEASES 

being  Bcraped  along  the  ground  in  walking.  On  the  other 
hand,  an  element  of  instability  is  frequently  superadded, 
giving  rise  to  the  spastic-ataxic  gait,  which  is  so  commonly 
seen  in  this  disease. 

Sensory  system.  Subjective  symptoms  are  rarely  absent 
at  one  period  or  another.  Giddiness,  paresthesia,  numb- 
ness over  the  limbs,  body  or  face,  feelings  of  heaviness  and 
weight  in  the  limbs  are  common.  None  of  the  above  are 
painful.  The  only  painful  symptoms  to  which  such  patients 
are  liable  are  those  arising  from  muscular  spasms  of  the 
limbs.  Girdle  and  lancinating  pains,  such  as  are  common 
in  tabes,  are  strikingly  absent  in  disseminated  sclerosis. 
Headache  is  rare,  except  in  the  terminal  stages,  when  it 
may  be  associated  with  obstinate  constipation,  vomiting,  and 
sometimes  heniateniesis. 

Objective  sensory  signs  are  slight  and  rare  compared  with 
the  frequency  of  the  subjective  symptoms.  Careful  examina- 
tion, however,  will  seldom  fail  to  detect  slight  relative  losses 
of  cutaneous  sensibility  in  the  paresthetic  areas,  more 
especially  to  cotton  wool,  the  finer  degrees  of  heat  and  cold 
and  tactile  discrimination,  although  all  forms  of  sensibility 
may  be  slightly  impaired.  In  rare  cases  hemianesthesia  with 
hemiplegia  may  be  present.  Segmental  losses  of  sensation  are 
practically  unknown. 

Beflexes.  The  deep  reflexes  are  never  absent ;  on  the 
contrary  they  are  as  a  rule  increased,  and  where  motor  weak- 
ness is  present,  they  may  be  greatly  exaggerated,  and  clonus 
easily  obtained. 

The  superficial  reflexes  are  lost  or  diminished  over  the 
epigastrium  and  abdomen.  This  is  one  of  the  most 
characteristic  and  definite  signs  of  the  malady.  As  their  loss 
depends  upon  an  interference  with  the  fibres  of  the  pyramidal 
system  above  the  corresponding  segmental  levels,  they  will 
still  be  elicited  until  such  interference  has  occurred.  Hence, 
although  their  presence  does  not  exclude  the  existence  of 
disseminated  patches  elsewhere,  their  absence  indicates  in- 
volvement of  the  pyramidal  tracts. 

The  plantar  reflex  is  extensor  in  type  in  all  cases  where 
spastic  weakness  is  present.  As  a  rule  it  is  an  early  sign,  and 
may  persist  during  a  period  of  remission,  when  other  symp- 
toms have  disappeared.     Occasionally  in  the  early  stages  the 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    425 

response  may  be  flexor.  If  the  response  is  indefinite  on 
one  side,  and  the  corresponding  abdominal  reflex  is  absent, 
the  significance  is  that  of  an  extensor  response. 

Sphincters.  Some  degree  of  hesitancy  in  passing  water 
is  an  early  symptom,  especially  in  males  ;  in  females  the 
difficulty  is  in  retaining  it.  It  is  not  a  troublesome  symptom 
until  the  later  stages.     Constipation  is  very  common  ;  in  the 


Fig.  134. — Photograph  illustrating  permanent  hyperextension  of 
the  great  toes  in  a  case  of  disseminated  sclerosis. 


later  stages  this  may  give  rise  to  coprsemia,  which  may  be 
the  cause  of  death. 

The  sexual  functions  are  usually  impaired. 

Trophic  disorders  are  not  obtrusive,  but  vaso-motor  dis- 
turbances, such  as  sweating  and  oedema,  occasionally  occur. 
Bed-sores  may  develop  in  the  later  stages.  The  nails  may 
become  brittle. 

Clinical  types.  1.  Remittent  type.  This  type  is  char- 
acterised by  the  onset  of  slight  s3rmptoms,  such  as  numbness, 
motor  weakness,  diplopia,  temporary  loss  of  vision,  and 
sphincter  trouble.  Often  the  onset  may  be  determined  by  a 
shock,    illness,    or    accident,    and,    as   the    patient    recovers 


426  NERVOUS  DISEASES 

apparently  completely,  the  symptoms  are  attributed  to  hysteria. 
The  recurrence  of  symptoms  may  be  postponed  for  an  indefinite 
period,  even  up  to  five  or  ten  years.  As  a  rule  the  remission 
period  becomes  shortened,  the  attacks  last  longer,  and 
eventually  permanent  paralysis  develops,  and  the  typical 
manifestations  of  the  disease  ensue. 

2.  Paraplegic  type.  In  this  variety  the  early  symptoms 
are  referred  to  the  lower  limbs,  complaint  being  made  of 
heaviness,  numbness,  and  weakness  of  the  legs.  The  toes  tend 
to  scrape  the  floor,  flexor  spasms  of  the  legs  give  discomfort 
at  night,  and  there  is  difficulty  in  passing  water.  Gradually 
the  symptoms  increase,  and  the  typical  form  evolves  with 
symptoms  referred  to  implication  of  the  higher  portions  of 
the  nervous  system.  In  such  cases  inquiry  should  be  made 
as  to  a  previous  history  of  diplopia  and  transitory  paresis, 
which  are  not  connected  in  the  patient's  mind  with  his  illness. 
The  existence  of  nystagmus  in  these  cases  is  highly  suggestive 
of  disseminated  sclerosis. 

Although  the  motor  and  sphincter  paralysis  may  be  well 
marked,  segmental  loss  of  sensation,  as  in  compression 
paraplegia,  is  rare. 

3.  Hemi-  and  monoplegic  types.  In  these  the  symptoms 
are  of  sudden  onset  and  affect  one  limb,  a  portion  of  a  limb, 
or  one  side  of  the  body.  They  are  frequently  transient, 
and,  as  in  the  remittent  type,  may  be  suspected  of  being 
hysterical. 

4.  Cerebellar  type.  The  early  occurrence  of  patches  in 
the  cerebellum,  or  its  peduncles,  may  give  rise  to  cerebellar 
symptoms  without  any  affection  of  the  motor  or  sensory 
paths.  The  symptoms  are  mainly  hemiataxy  and  tremor, 
without  characteristic  changes  in  the  reflexes.  The  recogni- 
tion of  this  type  can  only  be  made  by  the  further  progress  of 
the  disease. 

In  whichever  of  these  ways  the  disease  may  commence, 
the  terminal  stages  present  similar  features  :  nystagmus, 
pallor  of  the  discs,  articulatory  speech  defects,  spastic  paresis 
or  paralysis  with  '  action  '  tremor,  either  universal  or  confined 
to  certain  portions  of  the  body,  some  degree  of  ataxia  and 
unsteadiness,  loss  of  sphincter  control,  incontinence  of  urine 
and  obstinate  constipation,  increased  tendon  reflexes  with 
clonus,  absence  of  the  abdominal  and  epigastric  reflexes,  and 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    427 

slow  typical  extensor  plantar  responses,  and  subjective  sensory 
symptoms  and  diminution  of  all  or  some  forms  of  cutaneous 
sensibility  in  irregular  areas.  In  this  condition  the  patient 
may  remain  bedridden  for  a  number  of  years,  and  in  the 
absence  of  bedsores  or  intercurrent  maladies  the  general 
health  may  be  well  sustained. 

Differential  diagnosis.  It  is  only  in  the  early  stages 
of  the  disease  that  a  difficulty  is  likely  to  arise  in  diagnosis, 
and  more  particularly  in  the  differential  diagnosis  from 
Hysteria.  The  onset  of  both  maladies  during  early  adult 
life,  the  tendency  shown  by  both  to  remission  and  relapse 
of  symptoms,  and  the  existence  in  both,  even  at  the  outset, 
of  paraplegic  symptoms,  often  accompanied  by  tremor  on 
volitional  effort,  present  an  ample  field  for  error. 

Difficulty  may  also  be  experienced  and  the  possibility  of 
error  considerable  in  the  diagnosis  of  disseminated  sclerosis 
from  Intracranial  Tumour,  situated  beneath  the  tentorium. 
In  all  these  disorders  nystagmus,  inco-ordination  of  move- 
ment, and  instability  may  be  early  and  well-marked  symptoms. 

It  is  also  important  to  distinguish  the  paraplegic  type  of 
disseminated  sclerosis  from  Paraplegia,  which  is  a  symptom 
of  many  other  conditions,  such  as  that  arising  from  the 
pressure  of  tumours  within  the  spinal  canal,  the  so-called 
primary  lateral  sclerosis,  and  the  '  ataxic  paraplegia  '  of  sub- 
acute combined  sclerosis. 

The  chief  points  in  the  differential  diagnosis  are  given  in 
the  table  on  p.  428. 

Prognosis.  Disseminated  sclerosis  is  a  progressive  dis- 
ease, terminating  in  paraplegia.  Death  in  the  majority  of 
cases  supervenes  upon  complicating  conditions,  such  as  cystitis 
and  pyelonephritis,  bedsores,  copraemia,  and  pulmonary  dis- 
orders. The  duration  of  life  varies  within  considerable  limits, 
even  after  the  disease  has  been  thoroughly  established. 

Cases  in  which  sphincter  paralyses  are  absent  or  only 
slight  have  been  known  to  last  for  many  years. 

The  marked  tendency  towards  a  remission  of  symptoms, 
particularly  in  the  early  stages,  enables  a  more  favourable 
outlook  to  be  given  in  those  cases  in  which  a  more  or  less 
complete  disappearance  of  the  symptoms  takes  place.  In  the 
remitting  type  of  the  malady,  the  intervals  of  remission 
diminish  in  duration  with  each  relapse,  until  the  time  comes 


428 


NERVOUS  DISEASES 


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GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    429 

when  the  phenomena  of  the  malady  persist.  Periods  of 
freedom  from  symptoms  have  been  known  to  last  for  five 
or  ten  years.  The  prognosis,  therefore,  in  any  given  case  has 
to  be  based,  partly  upon  the  type  of  the  malady  and  partly 
upon  the  absence  of  serious  complicating  features. 

Treatment.  Little,  if  any,  benefit  is  obtained  by  medicinal 
remedies  in  this  disease,  although  general  and  nerve  tonics, 
such  as  strychnine,  arsenic,  and  iron,  may  be  prescribed  with 
advantage.  For  the  sphincter  weakness,  ergot  and  belladonna 
often  prove  of  great  value. 

The  most  satisfactory  means  of  treating  the  disease  is  by 
massage,  passive  movements  of  the  limbs,  and  faradism.  In 
the  early  stages,  these  in  association  with  as  much  rest  as 
possible  and  the  avoidance  of  undue  fatigue,  are  usually 
sufficient  to  materially  relieve  the  symptoms.  In  the  later 
stages,  when  tremor  and  instability  are  present  in  addition  to 
motor  weakness,  rest  in  bed  should  be  enjoined.  Complica- 
tions should  be  treated  as  they  arise,  and  great  care  taken 
to  avoid  the  occurrence  of  cystitis,  bed-sores,  or  troublesome 
constipation.     Ataxia  may  be  treated  by  Frsenkel's  exercises. 

Some  types  of  the  disease  respond  very  satisfactorily  to 
massage  and  electrical  treatment.  In  these  cases  an  occa- 
sional course  of  treatment  is  often  of  great  value  in  delaying 
the  onset  of  complications,  or  diminishing  their  intensity 
should  they  be  present. 

Pregnancy  should  be  avoided,  as  the  disease  is  almost  in- 
variably aggravated  during  the  puerperal  period. 


Chapter  V 

SUBACUTE   COMBINED  DEGENERATION  OF 
THE   SPINAL   CORD 

This  disease  is  characterised  clinically  by  the  onset  of  sub- 
jective and  objective  affections  of  sensation  referred  to  the 
distal  portions  of  the  limbs,  followed  by  signs  of  interference 
with  the  motor  functions,  segmental  anaesthesias  and  sphincter 
paralyses,  and  terminating  in  flaccid  paraplegia,  loss  of   the 


430 


nervous  DISEASES 


tendon  reflexes,  muscular  atrophy,  mental   impairment,   and 
death. 

Etiology.  It  occurs  both  in  men  and  women,  but  rather 
more  frequently  in  the  latter.  It  commences  usually  between 
the  ages  of  forty-four  and  sixty-five,  and  is  not  infrequently 
associated  with  anaemia,   either  of  the  simple  or  pernicious 


Fig.  135. — Section  of  the  cervical  region  of  the  spinal  con 
subacute  combined  degeneration. 


from  a  case  of 


type.  It  is,  however,  far  from  clear  that  the  anaemic  state  of 
the  blood,  to  which  attention  has  been  drawn,  is  the  direct 
causal  factor.  More  probably  the  condition  is  due  to  some 
toxic  influence,  which  acts  both  upon  the  blood  and  upon  the 
nervous  system,  the  latter  suffering  not  only  directly,  but 
also  from  the  impoverished  condition  of  the  blood. 

Pathology.  Few  alterations  are  visible  in  the  nervous 
system  to  the  naked  eye.  In  some  cases  there  is  an  excess 
of  cerebro-spinal  fluid,  associated  with  slight  atrophy  of  the 
cerebral  convolutions.  On  section  of  the  spinal  cord,  areas  of 
degeneration  are  seen  especially  in  the  posterior  columns, 
around  the  periphery,  and  in  the  pyramidal  tracts.  The 
white  matter  around  the  central  grey  horns  stands  out  clearly 
in  contrast  to  the  surrounding  degeneration.  These  changes 
may  be  slight,  but  in  severe  cases  are  well  marked  in 
some  segments  and  encroach  more  closely  upon  the  grey 
matter.  In  most  cases  the  spinal  cord  is  pale  and  slightly 
atrophic. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    431 

Microscopically,  the  degeneration  is  usually  of  some 
standing  ;  and  although  some  nerve  fibres  stain  with  Marchi's 
osmium  bichromate  method,  the  degenerated  areas  are  more 
clearly  seen  after  staining  with  the  Weigert-Pal  hematoxylin 
stain. 

The  degeneration  is  always  most  marked  in  the  posterior 


Fig.  136. 


Fig.  137.     See  page  432. 


columns  and  around  the  periphery  of  the  cord.  In  old-standing 
cases  the  pyramidal  tracts  may  be  as  much  degenerated  as 
the  posterior  columns.  Stained  by  hematoxylin,  fuchsin,  and 
picric  acid  (Van  Gieson)  sections  show  an  absence  of  inflam- 
matory changes.  The  blood-vessel  walls  are  healthy,  but  in 
some  parts  the  arteries  are  engorged  with  blood,  and  in  other 
parts  they  are  empty ;    perivascular  cell  infiltration  is  also 


432 


NKKYOl'S  DISKASKS 


absent.  In  the  more  acute  cases,  rounded  hyaline-degenerated 
bodies,  staining  a  purple  colour,  are  seen  in  the  periphery  of 
the  cord  and  along  the  course  of  the  vessels,  especially  in  the 
posterior  columns.  The  neuroglial  cells  in  these  cases  are 
swollen,  often  vacuolated,  and  occasionally  degenerated ;  and 
as  a  rule  there  is  proliferation  of  the  neuroglial  nuclei  with 


Fig.  138. 


Pig.  L39. 

PlGS.  L36  wi>  L39. — These  sections  illustrate  tin-  characteristic 
changes  in  the  spinal  cord  from  a  ease  of  subacute 
con 1 1  lined  degeneration . 


some  swelling  of  the  surrounding  protoplasm.  In  more 
chronic  types  an  over-growth  of  the  neuroglia  may  be 
observed. 

In  the  degenerated  portions  of  the  cord  the  myeline  sheath 
of  the  nerve  fibres  is  broken  up  into  characteristic  globules. 
In  places  the  sheath  has  entirely  disappeared  and  the  axis 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    433 

cylinder   is    exposed  (Bielschovsky).     The   nerve    roots    are 
similarly  degenerated,  but  to  a  less  extent. 

Alterations  are  also  detected  in  the  nerve  cells,  more 
especially  in  the  cells  of  Clarke's  column,  which  show  a 
chromatolytic  formation  of  the  protoplasm.  In  the  anterior 
cornual  cells  the  changes  are  only  slight,  and  when  present, 


Fig.  140. — Section  of  the  cord  in  the  lumbar  region  from  a  case 
of  subacute  combined  degeneration. 


consist  of  an  aggregation  of  yellow  pigment  around  the 
periphery  of  the  cell ;  and  similar  changes  may  be  seen  in 
the  Betz  cells  of  the  cerebral  cortex  and  in  the  Purkinje  cells 
of  the  cerebellum.  These  cell  changes  are  always  slight  and 
by  no  means  universal.  On  the  other  hand,  the  changes  in 
the  nerve  fibres  are  out  of  all  proportion  to  those  seen  in  the 
nerve  cells. 

From  the  microscopical  examination  it  would  appear 
that  the  changes  are  neither  inflammatory  nor  vascular  in 
character,  nor  does  any  evidence  support  the  view  that  the 
alteration  is  due  to  a  primary  affection  of  the  nerve  cells.  It 
would  seem  rather  as  if  a  toxic  influence  was  at  work,  first 
upon  the  neurone  as  a  whole,  resulting  in  degeneration  of 
those  portions  of  the  fibre  farthest  removed  from  the  trophic 
influence  of   the  cell;  and  secondly  upon  the  blood,  which 

28 


434  NERVOUS  DISEASES 

becomes  impoverished  and  leads  to  malnutrition  of  the  nerve 
fibres  and  their  cells. 

Symptoms.  Although  the  term  'subacute'  was  applied  to 
this  disease  by  those  who  originally  defined  its  anatomy  and 
its  symptoms,  further  observation  and  the  study  of  a  larger 
number  of  cases  have  tended  to  broaden  its  conception. 

The  earliest  symptoms  are  referred  to  the  sensory  system, 
and  consist  of  paresthetic  sensations  of  numbness  and  coldness 
in  the  distal  parts  of  the  limbs.  As  a  rule  these  symptoms 
affect  the  feet  earlier  and  more  severely  than  the  hands,  and 
are  unaccompanied  by  any  obvious  circulatory  derangement. 
At  this  period  a  slight  diminution  to  all  forms  of  sensibility 
is  found  when  tested  objectively. 

The  first  stage  may  be  prolonged  for  several  years,  or  may 
temporarily  pass  away,  to  recur  within  a  period  of  a  few 
months  to  two  years.  More  commonly,  after  lasting  from 
three  to  eighteen  months,  it  gradually  merges  into  the  second 
stage. 

The  patient  now  begins  to  feel,  and  to  complain  of,  a 
sensation  of  heaviness  and  weakness  of  the  legs  and  arms  ; 
and  his  friends  may  notice  that  his  general  condition  is 
impoverished  and  his  appearance  is  white  and  anaemic. 
Spasticity  of  the  lower  limbs  gradually  develops,  its  onset 
being  heralded  by  the  presence  of  flexor  spasms  and  drawing 
up  of  the  legs  in  bed,  with  stiffness  and  cramps  in  the  muscles. 
This  spastic  state  may  increase  slowly  or  rapidly,  but  usually 
within  a  few  weeks  or  months  paraplegia  is  so  fully  developed 
that  walking  is  impossible. 

During  this  (second)  stage  the  general  condition  is  one  of 
ataxic  paraplegia,  not  limited  to  the  legs  alone,  but  affecting 
also  the  hands  and  arms.  Weakness  of  sphincter  action  is 
usually  present  some  time  during  this  period.  The  deep 
reflexes  are  exaggerated,  and  extensor  plantar  responses  with 
absence  of  abdominal  reflexes  are  always  noticed. 

Of  the  organic  signs,  the  state  of  the  abdominal  reflexes 
and  the  plantar  response  may  afford  the  earliest  evidence  of 
the  affection  of  the  motor  system,  and  definitely  precede  the 
onset  of  obvious  spasticity. 

During  this  period  also  the  objective  sensory  symptoms 
undergo  a  marked  change.  An  examination  reveals  a  loss 
of  sensation  up  to  a  definite  segmental  level.     Attendant  on 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    435 

the  development  of  this  segmental  anaesthesia,  paresthetic 
symptoms  of  tightness,  coldness,  or  numbness  may  be  present. 
It  is  of  interest  to  note  that  at  the  autopsy  a  greater  local 
incidence  of  the  pathological  changes  is  found  to  exist  in  the 
segment  corresponding  to  the  upper  limit  of  the  anesthesia. 
The    development    of    these    segmental    symptoms    is    not 


Fig.  141. 


Pig.  142. 


Fig.  143. 


Three  charts  illustrating  the  sensory  symptoms  in  subacute  combined  de- 
generation of  the  cord.  Fig.  141  shows  the  distribution  of  the 
parsesthesia  in  an  early  stage  of  the  disease  (dotted  areas).  Fig.  142 
shows  the  sensory  loss  confined  to  the  distal  portions  of  the  limbs 
(shaded  areas).  Fig.  143  shows  the  sensory  loss  below  a  segmental 
level  in  the  late  stages. 


infrequently  attended  by  a  rise  of  temperature  of  one  or  two 
degrees,  and  a  decided  deterioration  in  the  general  condition. 

The  occurrence  of  such  indications  of  segmental  lesion 
ushers  in  the  final  stage  of  the  malady.  Not  uncommonly 
these  exacerbations  recur  at  increasingly  higher  levels ;  and 
the  muscles  below  the  level  of  the  lesion  begin  to  waste  rapidly. 
In  a  few  weeks  the  spastic  paraplegia  gives  place  to  flaccidity 
with  loss  of  all  the  reflexes,  the  extensor  responses  being 
retained  when  all  the  others  have  disappeared. 

Loss  of  sphincter  control  becomes  complete,  and  hemorrhage 

2S  * 


436  XKRVors  DISEASES 

from  the  bladder  is  not  infrequent,  and  may  be  a  terminal 
complication.  Bedsores  develop,  the  mental  condition  deterio- 
rates rapidly,  drowsiness,  torpor,  or  mild  delirium  supervene, 
and  death  results  from  pulmonary,  bladder,  or  renal  com- 
plications, and  general  exhaustion. 

Such  in  brief  is  the  clinical  history  of  a  disease  which  has 
a  very  definite  clinical  and  pathological  basis,  and  by  its 
recognition  there  has  been  brought  into  the  clear  light  of 
knowledge  one  more  of  the  ill-defined  diseases  which  have 
sheltered  under  the  clinical  covering  of  'ataxic  paraplegia.' 

Aberrant  types  of  this  condition  are: — 

1.  Cases  in  which  the  first  stage  exists  for  over  three 
years  without  any  obvious  evidence  of  organic  change. 

2.  Cases  in  which  the  sensory  symptoms  are  early  and 
severe,  and  are  accompanied  by  pains  in  the  limbs.  In  these 
cases  the  spasticity  may  be  overshadowed  or  absent,  and  the 
deep  reflexes  abolished  in  an  early  stage. 

3.  Cases  in  which  spasticity  niay  coexist  with  only  slight 
subjective  sensory  symptoms  for  a  long  period.  This  is  an 
especially  frequent  type  in  older  people. 

Diagnosis.  This  malady  presents  so  many  phases  that,  if 
seen  for  the  first  time  in  any  one  of  its  stages,  it  may  readily 
be  mistaken  for  any  form  of  nervous  disorder,  organic  or 
functional. 

The  first  stage  has  to  be  distinguished  from — 

1.  Hysteria  (p.  522)  by  : 

(a)  Its  onset  over  the  age  of  thirty-five  years  and  the 

absence  of  any  stigmata  of  hysteria. 

(b)  The  existence  of  ansemia  of  recent  origin. 

(c)  The  development  of  an  extensor  plantar  response. 

2.  Acroparesthesia  associated  with  atheroma,  rheumatoid 
arthritis  and  the  climacteric  period,  by  the  definite  signs  of 
organic  disease,  especially  the  extensor  plantar  response. 

The  second  stage  has  to  be  distinguished  from — 
1.  Disseminated  sclerosis  (p.  418)  by: 

(a)  The  absence  of  nystagmus,  or  optic  atrophy. 

{b)  The  age  of  the  patient  and  a  history  of  freedom 

from  previous  symptoms  of  nervous  disease. 
(c)  The  course  of  the  disease,  the  limitation  of  the 
paresthesia  to  the   distal  extremities,   and   the 
development  of  segmental  anaesthesia. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    437 

id)   The  coincidental  occurrence  of  severe  anaemia. 

2.  Syphilitic  meningo -myelitis  (p.  374)  by  : 

(a)  Absence  of  a  history  and  the  signs  of  syphilis. 

{b)  The  retention  of  the  pupillary  light-reflex,  absence 

of  cranial  nerve  palsies,  and  the  occurrence  of 

paresthesia  in    the   hands,  above    a   segmental 

anaesthesia. 
(c)  The  absence  of  lymphocytosis  in  the  cerebro-spinal 

fluid. 

3.  Tabes  dorsalis  (p.  379).  The  only  likelihood  of  difficulty 
in  diagnosis  arises  in  the  sensory  form  or  in  the  terminal  stages, 
when  motor  symptoms  are  marked  by  flaccid  paraplegia  and 
the  deep  reflexes  are  absent.     The  points  of  difference  are : 

(a)  Absence  of  pupillary  and  cranial  nerve  changes. 

(b)  Coincidental  loss  of  all  forms  of  sensation  without 

special   affection  of   cutaneous  or  muscle  pain 
sensibilities. 

(c)  Presence  of  an  extensor  plantar  response. 

(d)  The  absence  of  lymphocytosis  in  the  cerebro-spinal 

fluid. 

4.  Peripheral  neuritis  (p.  137)  by : 

(a)  Absence  of  any  toxic  cause. 

(b)  Absence  of  tenderness  on  pressure  along  nerves. 

(c)  Presence  of  extensor  response. 

id)  Absence  of  trophic  changes  and  drop  foot  or  wrist. 

(e)  Absence  of  sphincter  trouble. 

Prognosis.  The  prognosis  is  most  unfavourable.  In  the 
senile  cases  the  duration  of  life  may  extend  for  several  years ; 
but  in  all  other  cases,  and  as  soon  as  the  segmental  anaesthesia 
develops,  the  duration  seldom  exceeds  two  or  three  months, 
but  in  each  case  the  condition  of  the  patient  and  the  rapidity 
of  the  onset  of  symptoms  must  be  considered  in  forming  an 
opinion. 

Treatment.  Little,  if  anything,  can  be  done  to  arrest 
the  progress  of  the  disease,  but  general  tonic  treatment,  an 
open-air  life,  attention  to  the  mouth  and  teeth,  and  iron, 
arsenic,  and  cod-liver  oil,  may  delay  the  progress  of  the 
early  stage.  Those  cases  in  which  a  remission  of  symptoms 
occurred  were  treated  in  this  manner. 


438  NERVOUS  DISEASES 

Chapteb    V  1 
ACUTE  POLIOMYELITIS 

(SYN.  :     IMAM1I.K    PARALYSIS,    ACDTE    ATROPHIC    PALSY    OF 
AIUI.TS,    A.CUTE    ANTERIOE    POLIOMYELITIS) 

This  is  an  acute  specific  disorder,  characterised  by  the 
onset  of  constitutional  symptoms  and  the  sudden  develop- 
ment of  paralysis,  resulting  either  in  recovery  or  in  permanent 
atrophic  paralysis. 

Etiology.  This  disease  is  as  common  in  males  as  in 
females,  and  affects  young  adults  as  well  as  children,  although 
it  is  more  frequent  amongst  the  latter.  It  is  by  no  means  so 
rare  in  adults  as  was  at  one  time  supposed. 

It  has  a  definitely  seasonal  incidence,  occurring  more 
frequently  in  the  late  summer  and  early  autumn  months, 
statistics  from  several  different  countries  showing  that  it  is 
most  common  during  the  months  of  June,  July,  August,  and 
September.  That  it  is  an  infective  disorder  is  shown  by  its 
tendency  to  attack  several  members  of  the  same  family  about 
the  same  time,  or  several  persons  working  in  the  same  locality. 
It  is,  moreover,  known  to  occur  in  epidemic  form,  several 
well- authenticated  instances  of  which  have  been  recorded. 

The  clinical  association  which  has  been  sometimes  found 
to  exist  between  poliomyelitis  and  encephalitis,  and  the 
similarity  of  the  pathological  changes,  would  point  to  an 
identity  between  these  conditions. 

Pathology.  The  pathological  changes  found  in  the  central 
nervous  system  in  cases  which  have  died  within  a  brief  period 
of  the  onset  are  characteristic.  They  are  not  confined  to  any 
particular  segment  or  portion  of  the  cord,  but  are  more 
intense  in  the  grey  matter,  and  implicate  more  or  less  the 
whole  of  the  bulbo-spinal  centres  from  the  oculo-motor  nuclei 
to  the  conus  medullaris. 

The  changes  characteristic  of  the  disease  at  an  early  period 
are  a  well-marked  and  widespread  proliferation  of  small 
cells  in  and  around  the  veins  and  capillaries.  These  cells, 
which  seem  to  resemble  lymphocytes  in  appearance  and 
structure,  infiltrate  the  walls  and  adventitial  coat  of  the  blood- 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    439 

vessels.  The  grey  matter  of  the  spinal  cord  is  mainly  the 
seat  of  this  perivascular  infiltration,  but  not  solely,  for  it  is 
also  observed  around  the  vessels  in  the  pia  arachnoid  and 
those  radiating  into  the  white  matter,  mainly  upon  the  antero- 
lateral aspect  of  the  cord. 

In  addition  to  these  perivascular  changes,  a  well-marked 
infiltration  of  cells  occurs  in  the  grey  matter,  more  particularly 
of  the  anterior  cornual  region,  but  not  exclusively  limited  to  it. 
According  to  Farquhar  Buzzard, l  they  are  both  neuroglial 
cells  in  various  stages  of  proliferation  and  disintegration,  and 
lymphocytes  similar  to  those  seen  in  the  perivascular  infiltra- 
tions. In  the  neighbourhood  of  the  most  pronounced  infiltra- 
tions, the  ganglion  cells  of  the  anterior  horns,  as  well  as  those 
of  Clarke's  column  and  the  posterior  horns,  are  found  in  various 
stages  of  disintegration.  All  grades  of  cell  change  are  present, 
from  mere  chromatolysis  to  conditions  in  which  it  is  difficult 
to  discern  even  the  outline  of  the  cell. 

The  cerebro-spinal  fluid,  withdrawn  during  life,  frequently 
presents  a  large  lymphocytosis ;  and  although  it  is  not 
uncommon  to  detect  in  it  organisms  resembling  the  pneu- 
mococcus,  there  is  no  clear  evidence  that  such  are  the  causal 
factors  in  the  production  of  this  malady. 

In  consequence  of  the  destruction  of  the  ganglion  cells  and 
the  neuroglial  tissues,  the  appearance  of  the  spinal  cord  in  old- 
standing  cases  of  acute  poliomyelitis  is  characteristic.  A 
marked  shrinkage  of  the  anterior  horns  and  of  the  antero- 
lateral region  of  the  cord,  and  an  almost  complete  disappear- 
ance of  ganglion  cells  in  certain  limited  localities,  are  seen. 
The  fibres  of  the  corresponding  anterior  nerve  roots  are 
atrophied  as  a  result  of  the  destruction  of  their  ganglion  cells. 

Symptoms.  The  onset  of  the  disease,  whether  in  children 
or  adults,  is  ushered  in  by  pyrexia,  malaise,  and  pains  in  the 
head,  limbs,  and  body.  Sometimes  it  is  accompanied  by 
vomiting,  convulsions,  and  delirium.  The  temperature  may 
rise  to  102  F.  or  103 : F.  at  the  onset, remain  at  100°  F.or  101°  F. 
for  a  few  days,  and  then  gradually  subside  to  normal  in  about 
a  week.  The  pains  are  frequently  severe,  are  generally  a 
prominent  symptom,  and  are  occasionally  accompanied  by 
stiffness  and  rigidity  of  the  muscles  of  the  back,  suggesting 

1  Farquhar  Buzzard,  Brain,  1907. 


140 


NKKVors  DISEASES 


the  onset  of  meningitis.  The  muscles  are  tender  to  pressure, 
and  thf  patient  complains  of  pain  on  passive  movements  and 
handling  the  limbs.  This  may  persist  for  some  weeks  after 
all  constitutional  symptoms  have  disappeared. 

Within  a  day  or  two  of  the  onset  of  the  constitutional 
symptoms,  a   flaccid  paralysis  is  observed,  either  generally 

distributed  or  confined  to 
one  or  other  part  of  the 
body,  such  as  an  arm 
or  leg,  the  back  and 
abdomen,  and  in  rare 
cases  the  eyes  and  eyelids. 
During  this  early  period 
a  temporary  retention  of 
urine  is  not  uncommon. 

After  the  first  week, 
by  which  time  the  pyrexia 
and  constitutional  phen- 
omena have  disappeared, 
the  appearance  of  the 
patient  is  usually  suffi- 
ciently characteristic  of 
the  malady.  One  limb 
may  be  completely  para- 
lysed, and  the  others 
escape  or  be  partially 
affected,  or  there  may  be 
weakness  of  the  spinal, 
abdominal,  and  inter- 
costal muscles.  In  the 
rarer  cases  of  superior 
poliomyelitis,  ptosis,  and  ophthalmoplegia  externa  may 
be  observed,  with  or  without  difficulty  in  swallowing, 
anarthria  and  facial  palsy. 

In  all  cases  of  acute  poliomyelitis,  whatever  may  be  the 
part  of  the  central  nervous  system  permanently  affected, 
the  initial  paralysis  is  always  more  extensive  and  more 
complete  than  the  residual  or  permanent. 

Motor  system.  On  examination  of  the  motor  system, 
paralysis  of  wide  or  limited  extent  may  be  found.  The 
incidence  of  greatest  paralysis  may  be  in  the  limbs,  or  the 


Fig.  144, 


-A  case  of  extensive  acute 
poliomyelitis. 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    441 

trunk,  more  rarely  in  the  facial  and  ocular  muscles.  One 
feature  is  characteristic  of  the  disease,  namely,  that  at  the 
points  of  greatest  paralysis  the  distribution  of  the  paralysis 
is  according  to  the  nuclear  grouping  of  the  muscles  in  the 
spinal  cord.  Thus,  it  is  common  to  find  extensive  paralysis 
with  atrophy  affecting  one  group  of  muscles,  the  neighbouring 
muscles  remaining  intact.  For  example,  in  the  upper  limb, 
paralysis  of  the  shoulder  muscles  may  be  present  without 
any  affection  of  the  forearm  or  hand  muscles ;  in  the  lower 


Fig.  145. — Photograph  of  a  child  with  extensive  acute  polio- 
myelitis involving  the  abdominal  muscles.  The  illustration 
shows  the  '  ballooning '  of  the  belly  on  attempts  to  raise 
the  head. 

extremity  paralysis  of  the  anterior  tibial  group  may  exist 
without  affection  of  the  sural  and  posterior  tibial  muscles. 

But  whatever  the  distribution  or  extent  of  the  paralysis, 
the  maximum  loss  is  seen  immediately  after  the  onset.  Both 
the  extent  and  severity  of  the  palsy  diminish  in  course  of  time, 
at  all  events  up  to  about  two  years. 

The  palsy  is  of  the  flaccid  atrophic  type. 

In  fat  children  the  atrophy  may  not  be  apparent,  owing  to 
the  thickness  of  the  subcutaneous  tissue,  but  on  feeling  the 
limb  the  true  condition  is  revealed. 

The  effects  of  the  atrophic  palsy  are  shown  in  the  early 
stages  by  a  loss  of  movement  and  a  flail-like  condition  of  the 
limbs  with  relaxation  of  the  joints. 

Electrical  reactions.  All  degrees  of  the  reaction  of  de- 
generation may  be  seen,  from  the  fully  developed  reaction 
of  degeneration  to  a  mere  diminution  of  faradic  excitability. 
These  changes  begin  in  from  four  to  ten  days  after  the 
onset  of  paralysis. 


442 


NERVOUS  DISEASES 


As  time  goes  on,  deformities  may  develop.  They  arise 
in  two  ways:  either  by  contraction  of  the  non-paralysed 
muscles  owing  to  the  absence  of  the  opposition  of  the  paralysed 
muscles,  or  by  subsequent  contracture  of  the  paralysed  muscles. 
Spinal  curvature  may  develop  in  consequence  of  weakness 
of  the  spinal  muscles,  or  from  a  habitual  postural  attitude 
adopted  by  the  patient  to  obviate  a  locomotory  difficulty. 

Sensory  system.  Pain 
is  induced  on  handling  the 
muscles  or  moving  the 
limb.  This  may  be  a 
prominent  symptom,  and 
may  in  children  prevent 
the  discovery  of  the  par- 
alysis. Objective  loss  is 
exceptional,  but  slight 
degrees  of  impairment  of 
cutaneous  sensibility  have 
been  detected  in  a  few 
cases  during  the  early 
stages    and     are      always 


transient. 
Reflexes. 

reflexes    are 


The    tendon 
abolished  in 


Fig.  146. — Shows  wasting  of  the  left 
arm  in  an  old  case  of  acute  polio- 
myelitis. 


the  paralysed  muscles. 
During  the  earlier  stages 
an  increase  of  the  deep 
reflexes  below  the  affected 
segments  may  be  observed. 
Thus,  in  one  case  in  which 
there  was  extensive  paralysis  of  the  upper  extremity,  the  deep 
reflexes  on  the  corresponding  leg  were  definitely  increased, 
in  association  with  an  extensor  plantar  response.  These 
signs  usually  disappear,  and  are  to  be  attributed  to  slight 
interference  with  the  pyramidal  tract  during  the  initial 
widespread  inflammatory  condition  of  the  cord. 

The  integrity  of  the  superficial  reflexes  depends  upon  the 
state  of  the  reflex  arc  and  muscles. 

Sphincters.  Retention  of  urine  is  common  in  the  early 
stages,  and  weakness  of  the  abdominal  muscles  may  cause  a 
slight  difficulty  both  of  micturition  and  defecation.     Where 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    443 


the  lesion  has  affected  the  lumbosacral  enlargement,  dribbling 
of  urine  may  persist  for  a  time. 

Trophic  changes  may  be  confined  to  the  muscles.  In 
severe  cases  the  growth  of  the  limb  may  be  impaired. 

Diagnosis.  The  points  of  differential  diagnosis  between 
acute  poliomyelitis,  Landry's  paralysis,  and  toxic  polyneuritis 
are  given  on  p.  448. 

Prognosis.  The  prognosis  of  this  malady  has  to  take 
into  consideration  two  eventu- 
alities :  first,  the  likelihood 
of  recovery  from  the  intensity 
of  the  initial  symptoms  ;  and, 
secondly,  the  degree  and  ex- 
tent of  the  permanent  paraly- 
sis and  deformity. 

In  the  first  place,  there 
is  a  severe  and  fulminating 
type  of  the  disease,  in  which 
paralysis  is  extensive  in  dis- 
tribution and  of  rapid  onset, 
leading  to  death  within  a  few 
days.  This  is  a  rare  type, 
but  one  which  requires 
recognition,  as  it  has  not 
uncommonly  been  mistaken 
for  Landry's,  or  acute  as- 
cending paralysis. 

In  the  majority  of  the 
cases  of  acute  poliomyelitis 
the  constitutional  symptoms 
subside  satisfactorily,  and  the 
patient  enters  upon  a  long  period  of  slow  recovery,  with  more 
or  less  paralysis  and  disablement.  On  the  other  hand,  he 
may  almost  completely  recover. 

What  amount  of  paralysis  is  likely  to  remain  may  to  some 
extent  be  based  upon  the  electrical  reactions.  Those  muscles 
which  show  the  complete  reaction  of  degeneration  from  three 
to  six  months  after  the  onset  remain  paralysed.  The  degree 
of  atrophy  also  is  of  value  in  gauging  to  what  extent  paralysis 
of  individual  muscles  may  recover. 

Treatment.     The  treatment  varies  according  to  the  stage 


Fig.  147. — Shows  eversion  of  the  foot, 
in  an  old  standing  case  of  acute 
poliomyelitis,  from  paralysis  of  the 
posterior  tibial  muscle  (talipes 
valgus). 


444  NERVOUS  DISEASES 

at  which  the  patient  comes  under  observation.  In  the  acute 
or  pyrexia]  period  treatment  should  be  upon  general  lines, 
rest  in  bed,  light  diet,  and  sedative  draughts  for  the  relief  of 
the  pains,  restlessness,  and  malaise. 

After  the  first  week,  when  the  paralytic-  symptoms  have 
become  apparent,  hypodermic  injections  of  strychnine,  galvano- 
faradism  to  the  paralysed  muscles,  and  massage  ought  to  be 


Fig.  1-iS. — Talipes  calcaneus   in   a   case    of   acute   poliomyelitis 
from  paralysis  of  the  calf  muscles. 


commenced  and  continued  for  several  months.  It  is  most 
important  that  the  paralysed  limb  or  limbs  should  be  kept 
warm ;  hence  the  constant  use  of  warm  woollen  stockings  or 
bed  slippers  is  desirable. 

The  method  of  applying  the  electrical  treatment  is 
important.  In  all  cases  an  electrical  examination  of  the 
muscles  ought  to  be  made,  with  a  view  to  ascertain  the 
degree  and  extent  of  the  degenerative  atrophy.  To  those 
muscles  which  fail  to  respond  to  the  faradic  current, 
galvanism  ought  to  be  applied,  while  the  faradic  current 
should  be  used  to  those  in  which  the  faradic  excitability  is 
still  present.  The  combined  current  is  best  and  most  satis- 
factory for  this  purpose.     As  soon  as  voluntary  power  begins 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    445 


to  return,  the  patient  should  be  encouraged  in  the  use  of 
exercises  and  of  movements  against  slight  and  graduated 
resistance. 

It  not  infrequently  happens  that  during  the  early  stages 
non-paralysed  muscles  are  used  in  place  of  those  that  are 
paralysed,  and  in  the  later  stages,  when  recovery  has  taken 
place,  the  child  neglects  to  bring  them  back  into  use.  This 
may  be  overcome  by  en- 
couraging the  patient  in  the 
physiological  use  of  the  pre- 
viously paralysed  muscles,  or 
by  means  of  an  apparatus. 

Deformities  should  be 
prevented  by  judicious 
manipulations  or  by  the  ap- 
plication of  apparatus  at 
those  joints  where  the  over- 
action  of  unopposed  muscles 
is  likely  to  cause  distortion. 
If,  in  the  later  stages,  de- 
formities have  already  devel- 
oped, special  appliances  may 
be  used,  or  the  question  of 
tenotomy  may  require  con- 
sideration. Special  supports, 
such  as  a  poroplastic  jacket, 
are  necessary  in  those  cases 

in  which  the  trunk  muscles  are  permanently  paralysed 
149.) 

Two  operative  procedures  have  been  recommended  with  a 
view  to  overcome  or  minimise  the  effects  of  paralysis  when 
limited  to  a  particular  group  of  muscles — tendon  trans- 
plantation and  nerve  anastomoses.  The  one  method  brings 
the  paralysed  muscle  back  into  use  by  attaching  to  it  a 
portion  of  the  tendon  of  an  adjoining  healthy  muscle,  the 
other  by  anastomosing  its  nerve  to  an  adjacent  healthy  nerve- 
trunk. 

Progress  is  often  slow,  little  improvement  being  observed 
even  from  month  to  month.  Treatment,  more  especially  by 
means  of  massage,  movements  against  resistance  and  exer- 
cises, should  be  persevered  with  for  at  least  eighteen  months. 


Pig.  149. — Photograph  of  a  case  of 
acute  poliomyelitis,  affecting  the 
muscles  of  the  back,  and  showing 
a  resulting  spinal  curvature. 


(Fig. 


446  NERVOUS  DISEASES 

Ohapteb  VI 1 
LANDRY'S  PARALYSIS 

(syn.  :    MTTK    ASCENDING    PARAXTSIs) 

This  is  an  acute  or  subacute  disease,  characterised  by 
rapid  and  progressive  motor  paralysis  of  the  flaccid  type, 
without  muscular  atrophy  or  electrical  changes,  often  com- 
mencing in  the  lower  limbs  and  extending  upwards  to  involve 
the  trunk,  arms,  diaphragm,  and  cranial  nerves,  sometimes 
resulting  in  recovery,  but  usually  terminating  in  death  from 
respiratory  paralysis. 

It  is  more  common  in  males  than  in  females,  and  occurs 
mainly  between  the  ages  of  twenty  and  forty. 

Pathology.  The  morbid  changes  are,  according  to 
Farquhar  Buzzard,  almost  entirely  confined  to  the  ganglion 
cells  of  the  spinal  cord.  On  examination  by  Nissl's  method, 
early  chromatolysis  with  eccentration  of  the  nucleus  of  the 
cells  of  the  anterior  horns  and  of  Clarke's  column  is  observed. 
By  aid  of  Marchi's  osmium  bichromate  method,  small  fat 
droplets  are  found  in  the  fibres  of  the  spinal  cord  and  peri- 
pheral nerves.  These  changes  are  considered  by  Buzzard 
to  be  due  to  a  toxic  action,  as  they  are  common  to  other 
toxic  conditions.  Vascular  and  neuroglial  changes  are  absent, 
and  beyond  general  vascular  engorgement  no  macroscopic 
changes  are  noted. 

Various  micro-organisms   have  been   found,  but  it  is  not 
yet  possible  to  ascribe  the  disease  to  any  particular  form. 

Symptoms.  The  onset  is  insidious,  and  may  be  attended 
by  general  malaise,  headache,  a  feeling  of  fatigue,  and  by 
cramps,  pains,  and  tingling  in  the  legs  and  back.  Occasionally 
there  is  a  slight  rise  of  temperature,  rarely  exceeding  100°  F., 
sometimes  accompanied  by  anorexia.  These  premonitory 
symptoms  may  be  slight,  and  the  patient's  attention  is 
usually  first  attracted  by  a  feeling  of  tiredness  and  heaviness 
in  the  legs,  which  rapidly  become  weakened  and  eventually 
paralysed.  One  leg  may  be  affected  before  the  other,  but 
generally  the  weakness  is  present  in  both,  although  pre- 
dominating   in    one.     The    paralysis    does    not    invariably 


GENERAL  DISEASES  OF  THE  NERVOUS  SYSTEM    447 

commence  in  the  legs,  and  we  have  Been  cases  in  which  it 
originated  in  the  arms.  Cases  also  are  on  record  in  which 
paralysis  of  the  motor  cranial  nerves  was  the  first  symptom. 

The  motor  weakness  may  affect  the  proximal  portions 
of  the  limbs,  such  as  the  hip  and  shoulder  muscles,  before 
it  affects  the  distal  portions.  But  in  whatever  manner  it 
commences,  the  condition  rapidly  spreads,  and  in  the  common 
or  leg  type  the  paralysis  of  the  lower  limbs  is  such,  that 
within  six  to  twenty-four  hours  the  patient  is  unable  to 
walk,  and  the  legs  are  in  a  condition  of  extreme  flaccid 
paralysis  with  loss  of  the  deep  reflexes.  The  trunk  and 
thoracic  muscles  gradually  become  weak,  so  that  the  patient 
is  unable  to  sit  up  and  the  respiration  becomes  diaphrag- 
matic. In  a  few  hours,  or  in  three  or  four  days,  the  palsy 
may  be  widespread.  The  patient  now  lies  propped  up  in  bed, 
with  the  arms  and  legs  in  a  state  of  complete  flaccid  palsy. 
He  is  unable  to  raise  his  head  from  the  pillow.  Respiration 
is  rapid  and  laboured,  the  intercostal  muscles  are  paralysed, 
the  diaphragm  acts  feebly,  and  the  accessory  muscles  are 
brought  into  action.  As  he  is  unable  to  cough,  mucus  accumu- 
lates in  the  air  passages.  Phonation  is  weak,  articulation 
and  mastication  feeble,  but  swallowing  may  still  be  possible. 
The  face  is  cyanosed,  sweating  is  profuse,  and  the  pulse  rapid 
(120-130  per  minute). 

The  mental  condition  remains  clear  throughout ;  the  patient 
is  unable  to  realise  the  seriousness  of  his  condition ;  he  feels 
well  and  can  give  an  accurate  account  of  his  symptoms. 

Cranial  nerves.  The  motor  nerves  alone  suffer,  chiefly 
the  fifth,  seventh,  ninth,  tenth,  eleventh,  and  twelfth ;  less 
commonly  the  oculo-motor  nerves  are  affected.  Complete 
palsy  of  the  cranial  nerves  is  never  observed,  and  the  pupils 
respond  to  light. 

Motor  system.  The  motor  weakness  is  more  or  less 
general,  the  distal  parts  of  the  limbs  not  suffering  more 
than  the  proximal,  as  in  toxic  polyneuritis.  When  the 
paralysis  commences  in  the  lower  limbs,  these  may  be  in  a 
state  of  profound  flaccidity  before  the  higher  parts  of  the 
body  are  completely  involved.  The  muscles  are  not  atrophic, 
and  do  not  show  any  atrophy  even  after  recovery.  Myotatic 
irritability  is  diminished,  but  beyond  a  slight  sluggishness  to 
faradism,  no  changes  in  the  electrical  reactions  are  observed. 


448 


NERVOUS  DISEASES 


Sensory  system.  Numbness  and  tingling  may  be  complained 
of  in  tbe  early  stages,  but  are  rarely  severe.  Pain  in  the  back 
and  cramps  in  the  legs,  with  slight  muscular  hyperesthesia  on 
pressure,  are  more  common,  but  never  approach  the  acuteness 
found  in  peripheral  neuritis.  Slight  objective  blunting  of 
cutaneous  sensibility  has  been  described,  but  as  far  as  per- 
sonal observation  goes,  its  presence  is  exceptional. 

Reflexes.  The  deep  reflexes  are  abolished,  the  superficial 
absent,  and  the  plantars  are  either  absent  or  flexor  in  type. 

The  sphincters  are  not  affected,  except  in  so  far  as  the 
patient,  partly  from  the  position  in  bed,  and  partly  from  palsy 
of  the  abdominal  muscles,  has  a  difficulty  in  passing  urine. 

Course  and  prognosis.  In  acute  cases  death  may 
occur  in  from  two  to  six  days,  but  the  disease  may  still  prove 
fatal  even  after  three  or  four  weeks.  If  the  paralysis  ceases 
to  increase  during  a  space  of  twenty-four  hours,  the  outlook 
as  regards  life  is  materially  improved,  but  the  majority  of 
cases  end  fatally.  If  death  does  not  ensue,  the  prognosis  as 
regards  recovery  of  motor  power  is  quite  good,  and  there  is 
no  liability  to  relapse. 

Treatment.  The  patient  should  be  placed  at  rest  upon 
a  water-bed.  Diaphoresis  should  be  encouraged.  The  with- 
drawal of  cerebro-spinal  fluid  by  lumbar  puncture  is  advan- 
tageous. Oxygen  relieves  the  distress  of  the  later  stages, 
and  strychnine  and  alcohol  may  be  given. 


Differential  Diagnosis  between  Poliomyelitis,  Landry's  Paralysis, 
and  Acute  Polyneuritis 


Poliomyelitis. 

Landry's 
Paralysis. 

ACDTE 

Polyneuritis 

Age  at  onset 

Childhood 

Adults 

Adults 

General 
symptoms 

Well-marked 

Slight 

Often  absent 

Onset  of  palsy 

Sudden 

Gradual 

Gradual 

Distribution 

General  or  accord- 
ing to  nuclear 
grouping 

Lower  limbs,  ex- 
tending       up- 
wards 

Peripheral 

Effects  of 
pressure 

Muscles  tender 

No  tenderness 

Nerves  tender 

Sensory 
symptoms 

Pare 

None 

Of  peripheral  type 

Part    X 
VASO-MOTOK  AND  TROPHIC  DISEASES 

RAYNAUD'S  DISEASE.     SYMMETRICAL   GANGRENE 

This  disorder  is  characterised  by  paroxysmal  and  recur- 
rent attacks  of  transitory  interference  with  the  blood  supply, 
resulting  in  local  syncope,  asphyxia,  or  gangrene,  and  affecting 
as  a  rule  the  extremities,  usually  bilateral  and  symmetrical 
in  distribution,  and  always  associated  with  spasm  of  the 
arterioles. 

Etiology.  It  is  more  common  in  women  than  in  men, 
and  more  frequent  in  young  adults  than  in  the  old.  It  is 
more  often  seen  in  persons  of  nervous  disposition,  especially 
when  run  down  or  enfeebled.  Exposure  to  cold,  especially 
when  produced  by  a  sudden  drop  of  temperature,  is  the 
most  important  of  the  exciting  causes.  The  disease  also 
shows  a  certain  periodicity,  and  in  women  is  worse  during 
the  menstrual  period. 

Pathology.  This  is  not  definitely  known,  but  the 
symptoms  appear  to  have,  as  an  underlying  factor,  distur- 
bance of  the  vaso-motor  system,  manifested  by  arterial 
spasm,  which  may  be  induced  reflexly  by  cold  or  emotional 
shock.  There  is  no  constant  or  well-recognised  morbid 
anatomy,  but  changes  in  the  peripheral  nerves,  and  in  the 
later  stages  of  some  cases  obliterative  arteritis  have  been 
found. 

Symptoms.  Three  degrees  or  phases  of  the  disease  have 
been  described. 

(a)  A  mild  form,  or  local  syncope.  In  this  variety  com- 
plaint is  made  of  a  feeling  of  deadness  and  numbness  of 
the  fingers,  which  rapidly  become  pale  and  blanched,  with 

29 


450  NERVOUS  DISEASES 

lowering  of  the  surface  temperature.  The  condition  is 
usually  symmetrical,  and  affects  the  upper  and  lower 
extremities.  It  may  he  limited  to  the  ringers  and  toes,  or 
may  involve  the  whole  of  the  hand  or  foot. 

Stiffness  of  the  fingers  and  toes,  with  some  disturbance  of 
sensibility,  are  observed.  The  objective  sensory  loss  consists 
of  impairment  of  tactile  sensibility  and  a  more  marked  degree 
of  analgesia.  There  is  never,  however,  any  complete  dissocia- 
tion of  sensation.  Deep  sensibility  is  not  impaired.  Owing 
to  the  stiffness  and  sensory  defects,  the  patient  is  unable  to 
execute  fine  movements  of  the  fingers,  but  the  grasp  may  be 
relatively  unimpaired. 

During  an  attack,  which  may  last  from  a  few  minutes  to 
one  or  two  hours,  the  pulse  is  small,  but  is  never  obliterated. 
In  the  mildest  forms  a  rapid  return  to  the  normal  takes  place 
without  much  reaction  ;  but  in  the  graver  forms  the  return 
is  marked  by  burning  pain,  tenderness  on  pressure,  flushing, 
and  sometimes  local  perspiration. 

(b)  A  moderate  form,  or  local  asphyxia.  The  initial  stage 
of  this  variety  consists  of  a  slight  blanching  of  the  extremities, 
which  rapidly  gives  place  to  local  asphyxia,  commencing 
in  the  tips  of  the  fingers  or  toes,  and  spreading  upwards  to 
involve  the  hands  and  feet,  but  rarely  extending  above  the 
elbows  or  knees.  This  condition  may  also  affect  the  point  of 
the  nose  and  the  lobes  of  the  ears.  The  colour  of  the  parts 
varies  from  a  purple-red  to  a  slate  or  blue-green.  The 
nails  become  dark,  the  veins  swollen,  and  the  surface  tempera- 
ture is  much  reduced.  A  slight  cedema  is  usually  present,  and 
on  pressure  a  white  spot  is  left,  which  persists  for  a  consider- 
able time.  The  stiffness  and  the  sensory  changes  are  more 
pronounced  than  in  the  syncopal  form. 

The  duration  of  these  attacks  varies  from  a  few  hours  to 
two  or  three  days,  and  the  reaction  is  more  pronounced,  being 
attended  by  pain,  local  perspiration,  tenderness,  and  occasion- 
ally by  puffiness  or  powdery  desquamation. 

(c)  A  severe  form,  or  local  gangrene.  In  this  form  trophic 
changes  of  a  permanent  character  take  place,  resulting  from 
an  increase  either  of  the  local  syncope  or  of  the  asphyxia. 
The  pain  is  severe,  and  small  vesicles,  which  burst  and 
discharge  blood-stained  fluid,  form  over  a  limited  area.  The 
underlying  skin  dies,  and  a    small  sequestrum  of  necrosed 


VASO-MOTOIl  AND  TROPHIC  DISEASES         451 

tissue  is  formed,  bounded  by  a  zone  of  ulceration.  The  bone 
is  rarely  affected. 

Complications.  Cerebral  complications  of  the  nature  of 
epileptiform  fits  are  not  uncommon,  and  slight  degrees  of 
the  disease  are  often  associated  with  epilepsy.  Mental 
changes,  especially  acute  mania,  have  been  recorded  in  cases 
of  Raynaud's  disease.  Temporary  amblyopia,  coexistent  with 
vascular  changes  in  the  fundus  oculi,  have  also  been  noted  in 
rare  cases. 

Hemoglobinuria  or  hematuria  may  be  observed  either 
during  an  attack,  or  alternating  with  the  attacks  in  which  the 
extremities  are  affected.  This  complication  is  probably  more 
frequent  than  is  generally  realised. 

Diagnosis.  The  paroxysmal  character  of  the  attacks,  and 
the  absence  of  permanent  sensory  changes,  suffice  to  dis- 
tinguish this  disease  from  congenital  cardiac  disease,  syringo- 
myelia, and  senile  gangrene. 

It  may  be  distinguished  from  the  gangrene  associated 
with  endarteritis  obliterans,  by  its  symmetrical  distribution, 
its  superficial  extent,  the  absence  of  arterial  degeneration,  and 
the  preservation  of  the  pulse  in  the  affected  limbs. 

Somewhat  similar  conditions  are  seen  in  poisoning  by 
ergot  of  rye,  quinine,  and  the  coal-tar  products. 

Prognosis.  In  the  milder  forms  appropriate  treatment 
will  cut  short  an  attack,  and  care  and  attention  to  diet,  with 
avoidance  of  exposure  to  cold,  are  markedly  beneficial. 

In  the  gangrenous  form  the  prognosis  is  favourable,  so  far 
as  the  possible  spread  is  concerned,  and  even  if  amputation  is 
necessary  the  result  is  not  unsatisfactory. 

Treatment.  Attention  should  be  paid  to  the  avoidance 
of  cold  and  fatigue.  Cod-liver  oil  and  general  tonics,  regula- 
tion of  the  bowels,  attention  to  the  state  of  the  mouth  and 
digestion  are  important  in  lessening  the  tendency  to  the 
attacks.     Physical  exercise  should  be  encouraged. 

Galvanism  and  galvano-faradism  to  the  affected  limbs, 
applied  by  means  of  a  foot  or  hand  bath,  for  periods 
of  fifteen  to  forty  minutes,  may  check  or  cut  short  an 
attack.  Massage  of  the  limbs  is  also  important  and  useful 
for  this  purpose.  Submerging  the  hands  in  warm  or  hot 
water  during  an  attack  will  relieve  the  pain  and  discomfort. 

The  constant  use  of  warm  woollen  gloves  and  stockings  by 

29  * 


L52  NERVOUS  DISEASES 

those  who  are  subject  to  this  malady  is  recommended,  and  the 
avoidance  of  sudden  exposure  to  a  lowered  temperature. 


INTERMITTENT  CLAUDICATION.     INTBB  MITTBNT 

LIMPING 

This  is  a  condition  characterised  by  the  occurrence  of 
weakness,  paresthesia,  and  cramps,  mainly  in  the  legs,  which 
come  on  in  certain  persons  during  muscular  exercise,  and  is 
associated  with  angio-sclerosis  and  vaso-motor  disturbance. 

Etiology.  It  is  more  common  in  men  than  in  women, 
and  in  those  of  a  nervous  disposition.  Alcohol,  syphilis,  gout, 
a,nd  the  excessive  use  of  tobacco  and  tea  have  a  predisposing 
influence,  in  so  far  as  they  act  as  causes  of  vascular  degenera- 
tion. Aneurism  of  the  femoral  artery,  pressure  upon  the 
arteries  from  a  truss,  or  during  pregnancy,  have  been  found 
as  coexistent  features  in  several  cases.  The  association  of 
flat  foot  has  been  noted  in  a  number  of  instances. 

Pathology.  There  are  two  distinct  factors  in  the  pro- 
duction of  the  disease  :  (1)  an  angio-sclerosis  affecting  both  the 
arteries  and  the  veins  of  the  limb,  and  (2)  disturbance  of  the 
vaso-motor  mechanism.  The  latter  factor  alone  distinguishes 
the  cases  from  ordinary  senile  obliterative  endarteritis  with 
gangrene. 

The  vascular  changes  consist  in  thickening  of  the  intima 
of  the  arteries  and  the  veins ;  these  changes  are  constant  in 
the  small  vessels,  and  are  sometimes  associated  with  a  like 
process  in  those  of  larger  calibre. 

Slight  general  wasting  of  the  muscles  has  been  observed, 
and  degeneration  of  the  peripheral  nerves,  apparently  as  a 
secondary  result  of  the  sclerotic  changes  of  the  vasa  nervorum, 
has  been  described. 

Symptoms.  These  depend  upon  the  cutting  off  of  the  blood 
supply  to  the  muscles  and  nerves.  During  rest  no  discomfort 
is  present,  but  after  walking  for  a  short  time  the  leg  begins  to 
feel  heavy,  fatigued,  and  painful.  If  a  pause  is  made,  the 
symptoms  pass  away.  If,  on  the  other  hand,  the  patient 
persists  in  his  efforts,  the  leg  begins  to  feel  cold  and  a  pricking 
sensation  comes  on,  which  increases  to  a  feeling  of  numbness 
and   weakness,  with  sometimes  a  sensation  as  if  the  leg  is 


VASO-MOTOR  AND  TROPHIC  DISEASES  453 

going  to  burst.  The  muscles  become  stiff,  and  may  be  the 
seat  of  painful  cramp,  which  eventually  prevents  walking. 
The  foot  and  leg  may  appear  congested  and  swollen,  are 
usually  cold,  and  have  a  mottled,  cyanotic  look. 

On  examination  of  the  limb,  the  most  important  feature 
of  the  disease  is  detected,  namely,  obliteration  of  the  pulse  in 
the  dorsalis  pedis  and  posterior  tibial  arteries.  This  has 
been  observed  in  all  genuine  cases.  Although  the  condition 
is  usually  unilateral,  symptoms  may  affect  the  other  leg  to  a 
less  extent. 

Some  cases  progress  to  gangrene  of  the  foot.  In  this  way 
it  comes  about  that  clinically  this  affection  links  simple 
obliterative  gangrene  with  symmetrical  gangrene  or  Raynaud's 
disease. 

The  prognosis  is  bad,  but  by  prolonged  rest,  and  care 
in  avoiding  that  amount  of  exercise  which  will  induce  the 
pain,  the  symptoms  may  be  alleviated  and  the  progress  of 
the  malady  delayed. 

The  treatment  consists  in  avoiding  all  preventable  causes  of 
vascular  degeneration.  The  administration  of  iodides  and  of 
nitro-glycerine  are  of  little  value.  Locally,  warmth  obtained 
by  the  use  of  woollen  stockings  and  warm  baths  may  relieve 
the  symptoms  when  present ;  but  the  essential  feature  is  the 
curtailing  of  exercise  well  within  the  point  at  which  the 
symptoms  are  brought  on,  and  occasional  spells  of  rest  in  bed. 
We  have  found  considerable  relief  obtained  by  the  use  of  the 
high  frequency  currents,  and  gentle  massage  may  be  applied 
with  advantage. 

In  the  event  of  gangrene  supervening,  amputation  of  the 
limb  is  necessary. 

ERYTHROMELALGIA 

This  is  a  chronic  affection  characterised  by  the  occurrence 
of  severe  pain  usually  in  one  extremity,  followed  by  local 
redness  and  elevation  of  temperature  over  a  limited  area  of 
skin,  and  aggravated  by  a  dependent  position  of  the  limb  and 
muscular  exertion. 

Etiology.  It  occurs  in  persons  of  middle  life,  and  in 
men  more  commonly  than  in  women.  It  has  been  observed 
most    frequently    amongst    those    of    Jewish     descent.      A 


454  NERVOUS  DISEASES 

predisposing  cause  is  excessive  work,  with  long  periods  of  stand- 
ing ;  shock,  injury,  and  acute  diseases  have  been  cited.  It  has 
also  been  observed  in  association  with  some  forms  of  organic 
nervous  disease. 

Pathology.  It  depends  upon  disordered  vaso-motor  func- 
tions, combined  with  a  morbid  condition  of  the  blood- vessels, 
consisting  of  thickening  of  the  middle  and  inner  coats  of  the 
arteries,  and  in  some  cases  with  an  associated  degeneration  of 
the  peripheral  nerves. 

Symptoms.  It  is  most  common  in  the  lower  extremities, 
but  may  occur  elsewhere  ;  as  a  rule,  one  foot  only  is  affected. 
The  first  indication  is  pain  of  a  burning  character  coming  on 
towards  the  end  of  the  day  in  the  sole  of  the  foot. 

Rest  and  elevation  of  the  limb  may  bring  temporary  relief. 
In  course  of  time  the  pain  becomes  more  frequent,  and  is 
more  easily  induced.  Sooner  or  later  a  rosy  redness  appears 
in  patches,  especially  after  the  limb  has  been  dependent  for  a 
time.  The  affected  area  is  hot,  the  veins  engorged,  and  the 
arterial  pulse  may  be  exaggerated.  The  patch  becomes 
extremely  hypersensitive  to  deep  and  superficial  pressure,  and 
slight  swelling  may  be  seen.  The  pain  and  redness  are 
increased  by  heat  and  pressure,  and  relief  is  brought  about 
by  rest,  cold,  and  elevation  of  the  limb.  Slight  general 
muscular  wasting  may  develop  later. 

The  prognosis  is  bad,  the  affection  tending  to  become 
worse,  although  in  some  cases  it  has  disappeared  spontaneously. 

Treatment.  Gouty  or  syphilitic  taints  should  be  treated 
by  appropriate  remedies.  Eest,  elevation  of  the  limb,  and 
the  application  of  cold,  afford  temporary  relief ;  if  the  pain  is 
severe,  the  bedclothes  should  be  kept  off  the  feet  by  means 
of  a  cradle.  Section  of  a  nerve,  or  amputation  of  a  limb, 
has  not  proved  successful. 

FACIAL  HEMIATROPHY 

This  is  a  rare  condition,  characterised  by  atrophic  changes 
in  the  skin,  subcutaneous  tissues,  muscles,  and  bones  of  the 
face  upon  one  side  in  the  distribution  of  the  trigeminal  nerve. 

Etiology.  The  disease  usually  arises  before  puberty,  but 
only  rarely,  if  ever,  after  thirty  years  of  age.  It  is  more 
common    in    women  than  in  men.     In  a  few  cases  a  direct 


VASOMOTOR  AND  TROPHIC  DISEASES         455 

heredity  has  been  traced.  It  may  in  some  instances  be 
ascribed  to  acute  infective  diseases,  in  others  to  trauma,  but 
in  the  majority  no  obvious  cause  is  found. 

The  morbid  anatomy  consists  in  a  proliferating  inter- 
stitial neuritis  of  the  trigeminal  nerve  (Mendel),  but  it  is 
debatable  how  far  the  cutaneous  and  osseous  changes  are 
directly  and  solely  the  result  of  the  nerve  lesion.     If  this  were 


Fig.  150  is  a  photograph  of  a  case  of  left-sided  facial 
hemiatrophy. 

so,  it  is  difficult  to  understand  why  there  are  no  symptoms  of 
lesion  of  the  sensory  fibres,  which  form  the  bulk  of  the  fibres 
of  the  fifth  nerve.  It  is  more  probable  that  the  nervous  and 
cutaneous  lesions  arise  from  one  and  the  same  cause,  and 
are  not  cause  and  effect.  The  disease  would  appear  to  be  due 
to  an  arrest  of  development  during  the  growing  period,  arising 
from  a  morphcea  of  the  fifth  nerve  (Hutchinson). 

Symptoms.  The  skin,  usually  over  the  cheek,  chin,  or 
forehead,  becomes  white  or  whitish  yellow.  The  patch  thus 
formed  spreads,  sometimes  by  fusion  with  adjacent  patches. 
When  the  disease  is  well  marked,  there  is  a  characteristic 


456  NERVOUS  DISEASES 

depression  under  the  malar  bone,  arising  from  atrophy  of  the 
subcutaneous  tissue  in  this  locality.  If  the  change  involves 
the  hairy  parts  of  the  face,  alterations  in  colour  may  be  detected, 
or  the  hair  thins  and  falls  out.  The  facial  muscles  in  old- 
standing  cases  are  thinned  and  wasted,  but  do  not  show  the 
degenerative  changes  characteristic  of  nerve  lesion.  The 
facial  bones —frontal,  malar,  and  maxillary— also  become 
shrunken ;  the  nasal  cartilages  share  in  the  atrophic  change, 
but  the  ear  is  less  often  involved.  The  tongue  also  sometimes 
shows  atrophic  changes.     Sensation  is  unaffected. 

The  distribution  of  the  atrophic  change  on  the  face  varies, 
so  that  certain  varieties  have  been  described :  (a)  complete 
unilateral  facial  atrophy,  (b)  incomplete  unilateral  atrophy, 
(c)  bilateral  atrophy,  and  (d)  cases  with  similar  changes  in 
other  parts  of  the  body. 

Prognosis.  The  malady  is  progressive  and  has  no  ten- 
dency to  shorten  life. 

The  treatment  consists  in  the  administration  of  general 
and  nerve  tonics,  such  as  quinine,  iron,  arsenic,  and  strych- 
nine. Massage  of  the  facial  muscles  and  faradic  stimulation 
may  lessen  or  hinder  the  effects  of  the  atrophic  process. 

ANGIO-NEUROTIC  (EDEMA 

This  name  has  been  given  to  a  number  of  conditions 
characterised  by  localised,  or  general,  cedema  which  is 
unassociated  with  inflammatory  or  other  gross  lesion,  but 
generally  coexisting  with  some  impairment  or  alteration  of 
the  nervous  functions. 

Pathology.  It  belongs  to  the  same  class  of  angio- 
neuroses  as  Raynaud's  disease.  It  is  probably  dependent 
upon  a  local  disturbance  of  innervation  of  the  blood-vessels 
of  the  affected  area. 

Symptoms.  The  symptoms  consist  of  a  circumscribed 
swelling  and  cedema  of  the  skin  and  subcutaneous  tissues  in 
various  parts  of  the  body,  chiefly  of  the  face,  arm,  and  hand. 
The  swelling  appears  suddenly  without  obvious  cause,  and 
disappears  in  the  course  of  a  few  hours  or  days.  It  is 
unattended  by  pain  or  itching,  but  may  give  rise  to  a  tingling 
or  burning  sensation.  The  local  cutaneous  attacks  may  be 
accompanied   by  signs  of   similar  affection    of    the    mucous 


VASOMOTOR  AND  TROPHTC  DISEASES         457 

membranes,  such  as  vomiting,  diarrhcea,  dyspnoea,  and  even 
asphyxia  from  oedema  of  the  larynx  and  epiglottis.  Hsemo- 
globinuria  has  been  found  in  rare  instances.  It  has  a  special 
tendency  to  appear  in  certain  families. 

A  condition  known  as  hereditary  oedema,  which  probably 
stands  in  close  relation  to  the  angio-neurotic  form,  has  also 
been  described. 

Treatment  consists  mainly  in  the  administration  of 
general  and  nervine  tonics.  A  calomel  purge  at  the  onset  of 
an  attack  is  of  value,  and  nitro-glycerine  has  been  recom- 
mended should  the  seizures  be  frequent  or  persistent. 

SCLERODERMA 

This  is  a  localised  or  diffuse  hardening  of  the  skin,  affecting 
females  more  frequently  than  males,  and  occurring  during 
the  middle  period  of  life. 

Symptoms.  The  circumscribed  form  is  characterised  by 
the  appearance  of  hard,  brawny  patches  of  a  waxy  colour, 
developing  with  great  rapidity  and  disappearing  after  a  few 
weeks,  or  persisting  for  months  or  years.  It  is  most  common 
about  the  neck  and  chest.  In  the  diffuse  form,  a  brawny 
hardness  appears  in  the  skin  of  the  face  or  limbs,  and  gradually 
affects  the  whole  of  the  limb,  face,  or  trunk.  Occasionally  it 
is  universal  and  gives  rise  to  a  characteristic  appearance. 
The  face  is  expressionless,  eating  is  carried  out  with  difficulty, 
and  the  limbs  are  fixed  and  immobile. 

The  secretion  of  sweat  is  abolished,  the  skin  is  dry,  glossy, 
and  smooth,  and  sometimes  the  extremities  of  the  limbs 
present  a  cyanosed  appearance. 

Prognosis.  The  disease  is  chronic  and  may  persist 
for  many  years.  Death  occurs  from  pulmonary  or  renal 
complications. 

Treatment  consists  in  maintaining  the  body  temperature 
by  warm  clothing.  Warm  baths  and  massage  are  of  much 
use.     Rubbing  the  skin  with  oil  is  strongly  recommended. 


Part   XI 
FAMILIAL   DISEASES 

THE   MUSCULAR   DYSTROPHIES 

Grouped  under  the  title  of  Muscular  Dystrophies  are 
several  varieties  of  muscular  atrophy  in  which  the  voluntary 
musculature  is  primarily  involved,  and  the  nervous  system  is 
only  secondarily,  if  at  all,  affected. 

The  close  relationship  which  exists  between  the  nervous 
and  the  muscular  apparatus  renders  it  imperative  to  consider 
the  muscular  d}"strophies  along  with  the  diseases  of  the 
nervous  system,  as  they  give  rise  to  weakness  and  impairment 
of  movement,  and  have  to  be  distinguished  from  the  myelo- 
pathic muscular  affections,  or  those  secondary  to  disease  of 
the  nerve  elements. 

Although  muscular  d3Tstrophy  is  a  congenital  condition,  its 
manifestation  may  be  delayed  until  the  patient  has  advanced 
to  puberty,  adolescence,  or  later  life.  The  changes  in  the 
muscles  are  both  quantitative  and  qualitative.  The  former 
consist  of  defective  development  or  absence  of  certain  muscles, 
or  portions  of  muscles  ;  the  latter  of  a  potential  weakness  of 
the  muscle,  whereby  it  is  unable  to  develop  properly,  and 
sooner  or  later  becomes  the  seat  of  pathological  changes. 
These  alterations  may  give  rise  to  an  increase  in  the  bulk  of 
the  muscle  from  an  over-growth  of  the  interstitial  tissues — 
pseudo-hypertrophy,  or  to  a  diminution  and  atrophy  of  the 
muscle.  In  all  cases  the  tendency  is  eventually  towards 
muscular  atroph}7. 

The  muscular  dystrophies  have  been  divided  by  some 
writers  into  three  clinical  subgroups,  according  to  the  character 
and  situation  of  the  muscular  changes  ;  but,  although  many 

458 


FAMILIAL  DISEASES  459 

cases  fall  naturally  into  one  or  other  group,  there  are  some 
which  conform  to  none,  or  have  features  common  to  all. 


I.  Pseudo-hypertrophic  muscular  paralysis 

Etiology.  This  type  occurs  more  frequently  in  males  than 
in  females,  in  the  proportion  of  five  to  one.  When  present 
in  females  it  is  often  of  later  development  and  slighter  degree. 
Isolated  cases  are  rare,  the  disease  having  a  familial  distribution. 
It  is  always  transmitted  through  the  mother,  who  is  herself 
not  affected.  It  manifests  itself  during  the  period  of  develop- 
ment, either  in  late  infancy  or  in  mid  or  later  childhood.  The 
commonest  time  of  onset  is  between  the  ages  of  four  and  six, 
and  three- fourths  of  the  cases  occur  before  the  age  of  ten. 

Pathology.  In  the  pseudo-hypertrophic  forms  the  muscles 
are  pale  and  fatty-looking,  and  on  microscopic  examination 
an  increase  in  the  interstitial  substance  is  noted,  either  of 
fibrous  tissue,  of  fat,  or  of  both.  The  muscle  fibres  are 
thinned  and  irregular  in  shape,  large  in  one  portion,  small 
in  others.  Striation  is  often  indistinct,  and  the  fibres  may 
be  seen  undergoing  granular  degeneration,  hyaline  change, 
and  vacuolation.  The  nuclei  may  be  increased  in  number. 
In  some  cases  of  pseudo-hypertrophy  the  muscle  fibres  are 
enlarged  and  show  large  central  nuclei.  As  a  rule,  the  fibres 
are  best  preserved  where  there  is  an  increase  in  the  fatty 
rather  than  in  the  fibrous  interstitial  substance.  Bundles  of 
apparently  normal  muscle  fibres  may  be  scattered  through- 
out.     The  muscle  spindles  are  intact. 

Nervous  system.  Atrophic  changes  in  the  anterior  nerve 
roots,  consisting  in  a  loss  of  fibres  and  increase  of  fibrous 
tissue,  have  been  described. 

In  the  spinal  cord  itself  a  reduction  in  the  size  and  number 
of  the  anterior  cornual  cells  has  been  observed,  in  some  asso- 
ciated with  an  increase  of  the  neuroglial  matrix.  In  no  case 
has  any  degenerative  alteration,  either  of  nerve  cells  or  fibres, 
been  found  in  other  portions  of  the  central  nervous  system. 

Symptoms.  The  first  feature  which  calls  attention  to 
the  condition  is  usually  instability  in  walking,  the  child 
being  easily  knocked  over,  having  difficulty  in  going 
up  stairs,  and  showing  inabilit^y  to  play  games  like  other 
children.     Later  on,  the  gait  may  be  noticed  to  be  peculiar. 


460  NERVOUS  DISEASES 

This  disability  comes  on  gradually,  and  it  is  a  not  uncommon 
cause  of  wonderment  to  the  parents  why  a  child  with  such 
well-developed  legs  is  unable  to  walk  properly.  In  some  cases 
the  child  has  never  walked  well,  but  as  a  rule  it  has  been 
able  to  get  about  in  a  normal  way  for  some  months  or  a  year 
or  two  before  the  weakness  is  noticed. 

The  child  is  usually  fat  and  full-faced,  though  anoemic 
looking.  In  some  cases  the  muscular  hypertrophy  may  not 
be  at  first  apparent,  but  even  then  the  affected  muscles  can 
be  felt  to  have  a  hard,  *  putty '  feeling.  In  the  majority  of 
cases,  on  the  other  hand,  an  increase  of  size  in  certain  muscles 
is  at  once  obvious.  The  following  are  the  muscles  most  com- 
monly increased  in  size  : — 

Lower  limbs  :  Calf  muscles  ;  extensors  of  knee — rectus 
femoris  and  vastus  externus  ;  glutei  ;  anterior  tibial  muscles 
(rarely). 

Trunk  :  Lumbar  muscles. 

Upper  limbs  :  Supra-  and  infra-spinati ;  deltoid  ;  triceps 
and  biceps. 

Face  :  Masseters  (rarely). 

The  following  muscles  are  frequently  diminished  in  size, 
or  absent :  Lower  portion  of  the  pectoralis  major;  latissimus 
dorsi;  teres  major;  clavicular  part  of  the  sterno-mastoid. 

The  flexors  of  the  hip  are  also  usually  affected,  but  cannot 
be  objectively  examined. 

The  muscles  of  the  face  and  tongue,  forearms  and  hands, 
and  the  flexors  of  the  knee  are  not  affected.  As  the  disease 
progresses  the  implicated  muscles  gradually  atrophy,  so  that 
those  originally  enlarged  tend  after  a  time  to  become  small. 

Owing  to  the  bilateral  weakness  or  absence  of  the  lower 
part  of  the  pectoralis  major,  latissimus  dorsi,  and  teres  major, 
the  axillary  folds  are  practically  absent.  The  child  is  unable 
to  depress  the  arms  against  resistance,  and  it  is  impossible  to 
lift  the  child  up  by  the  arms.  Owing  to  the  weakness  of  the 
latissimus  dorsi,  coughing  is  very  feebly  performed. 

The  attitude  and  gait  are  characteristic.  The  child  stands 
on  a  wide  base  with  the  shoulders  and  upper  part  of  the  body 
held  well  back,  and  showing  a  marked  anterior  convex  curve 
in  the  lumbar  region.  This  lordosis  is  only  present  when 
the  patient  is  standing  erect,  and  is  the  device  made  use  of 
to  compensate  for  the  weakness  of  the  extensors  of  the  hips, 


FAMILIAL  DISEASES 


461 


as  by  its  aid  the  centre  of  gravity  is  thrown  further  back 
and  the  strain  upon  the  extensors  relieved.  In  walking,  the 
feet  are  lifted  off  the  ground  with  some  difficulty,  and  the 
pelvis  is  tilted  downwards  to  the  side  opposite  to  the  leg 
which  is  about  to  be  advanced.  As  the  advanced  leg  reaches 
the  ground  the  centre  of  gravity  is  transferred  to  it  by  tilting 
the  pelvis  to  the  same  side.     This  is  the  basis  of  the  waddling 


Fig.  151. 


Fig.  152. 


Figs.  151  and  152. — Illustrate  the  general  appearances  of  pseudohypertrophic 
muscular  paralysis. 


or  oscillating  gait  (alderman's  gait)  characteristic  of  pseudo- 
hypertrophic paralysis. 

The  full  demonstration  of  the  defect  is  best  seen  when  the 
patient  is  asked  to  rise  from  the  floor.  The  patient,  resting 
upon  his  hands  and  knees,  raises  the  lower  portion  of  the 
back  so  that  he  rests  upon  his  hands  and  toes,  placing  them 
as  far  apart  as  possible.  He  next  supports  some  of  the  weight 
of  his  body  with  one  hand  on  the  floor,  while  the  other  hand 
is  placed  upon  the  front  of  the  corresponding  thigh,  by  this 
means  aiding  the  weakened  extensors  of  the  knee.     He  now 


462  NERVOUS  DISEASES 

depresses  the  sole  of  the  foot  on  to  the  ground,  and  by 
gradually  working  the  one  hand  up  the  thigh,  at  the  same  time 
pressing  off  the  ground  with  the  other,  he  lessens  the  strain 
on  the  weakened  extensors  of  the  hip.  The  hand  which  still 
remains  upon  the  floor  is  now  placed  upon  the  thigh,  and  by 
gradually  working  both  hands  up  the  thighs  the  body  is 
brought  more  and  more  to  an  upright  position,  until  by  a 
final  effort  the  shoulders  are  thrown  back,  the  spine  curved 
forwards,  and  the  erect  attitude  attained.  In  more  advanced 
cases  the  patient  is  usually  unable  to  rise  at  all  from  the  floor. 

In  the  early  stages  the  lordosis,  present  when  standing,  is 
absent  in  the  sitting  posture  ;  but  in  advanced  cases  weak- 
ness of  the  erectores  spinas  results  in  curvature  when  sitting. 

Talipes  equinus,  due  to  the  fibrous  shortening  of  the  calf 
muscles,  is  one  of  the  earlier  deformities,  and  the  flexor 
contractures  at  the  knees  and  elbows,  resulting  from  the 
unopposed  action  of  the  flexors  of  the  knee  and  elbow  re- 
spectively, are  among  the  later. 

In  rare  cases  the  tongue  has  been  affected,  but  the  cranial 
nerves  and  the  sensory  system  remain  intact. 

The  electrical  examination  shows  a  quantitative  decrease 
to  faradic  and  galvanic  stimulation  corresponding  to  the 
degree  of  muscular  change. 

The  deep  reflexes  are  never  exaggerated,  but  become  im- 
paired as  the  various  muscles  atrophy,  and  are  finally  lost. 
Sphincter  trouble  is  rare. 

These  patients  are  as  a  rule  placid,  intelligent,  and 
good  tempered.  Mental  defect  is  not  common,  and  if  present 
ought  to  be  regarded  as  a  complication. 

2.  Cases  in  which  the  original  situation  of  the  muscular 
affection  is  similar  to  that  seen  in  pseudo-hypertrophy,  but 
in  which  no  hypertrophy  of  the  muscles  is  detected. 

3.  Cases  in  which  a  general  affection  of  the  muscular 
system  is  present. 

4.   Juvenile  muscular  atrophy 

This  is  an  affection  of  the  muscles  of  the  shoulder  girdle 
and  upper  arm,  usually  atrophic,  but  frequently  with  a 
combination  of  atrophy  and  pseudo-hypertrophy  (Erb's  type 
of  juvenile   muscular   atrophy).      The   typical   features   are 


FAMILIAL  DISEASES  463 

the  onset  of  the  malady  some  time  during  puberty  and 
early  adolescent  life,  usually  before  the  age  of  twenty  years.- 
The  muscles  of  the  shoulder  girdle  primarily  implicated  are 
the  lower  part  of  the  pectoralis  major  and  the  latissimus 
dorsi,  less  commonly  the  upper  part  of  the  pectoralis  major, 
trapezius,  serratus  magnus,  and  rhomboids.  In  the  upper 
arm,  the  biceps,  triceps,  and  supinator  longus  are  usually 
involved,  while  the  deltoid,  supra-  and  infra-spinati  as  a  rule 


Fig.  153. — A  case  of  Erb's  jurenile  muscular  atrophy  showing 
weakness  of  the  serratus  magnus  muscles  and  winging  of 
the  scapulae. 

escape,  but  in  some  instances  are  found  hypertrophied.  In 
the  pelvic  girdle  the  muscles  usually  affected  are  the  flexors 
of  the  hip  (ilio-psoas),  glutei,  and  the  extensors  of  the  knee. 
In  the  later  stages  the  trunk  muscles,  erecfcores  spinse,  inter- 
costals,  diaphragm,  and  abdominals  become  involved. 

5.  Cases  in  which  the  face  is  primarily  affected.  The  face 
may  be  affected  alone  in  infancy,  but  most  cases  develop  later 
into  the  facio-scapulo-humeral  type. 

6.   The  facio-scapulo-humeral  type 

This  is  a  type  in  which  the  face  is  primarily  involved,  and 
later  the  shoulder  and  upper-arm  muscles — the  facio-scapulo- 
humeral  or  Landouzy-Dejerine  type.  In  the  facio-scapulo- 
humeral  type    (Landouzy-Dejerine)    males  and   females   are 


464  NERVOUS  DISEASES 

affected  more  equally  than  in  the  pseudo-hypertrophic  form. 
It  is  more  constantly  present  in  successive  generations. 
Usually  the  onset  is  between  the  ages  of  fifteen  and  twenty- 
five.  The  muscular  changes  may  be  limited  to  the  original 
situation  for  a  number  of  years,  and  life  may  be  prolonged 
up  to  fifty  years  from  the  onset. 

It   is   characterised   by    an    affection   commencing   in   the 
facial    muscles — zygomatics,    orbicularis     oris,    risorii,    and 


Fig.  154. — Illustrates  the  appearance  of  the  face  and   1 ith  in 

the  tacio-scapulo-humeral  form  or  Lanclouzy-Dejerinc  type 
of  muscular  dystrophy. 

levatores  menti.  Occasionally  the  orbicularis  palpebrarum  is 
also  affected.  The  expression  of  the  face  is  more  or  less 
characteristic,  the  lips  are  everted,  and  may  remain  apart,  the 
lower  lip  protruding  and  giving  the  so-called  '  tapir  mouth.' 
The  lips  cannot  be  pursed,  or  applied  to  a  glass  in  drinking, 
whistling  and  blowing  out  the  cheeks  are  impossible.  Articu- 
lation is  affected  owing  to  an  inability  to  pronounce  labials. 
The  naso-labial  fold  is  lost,  and  in  smiling,  instead  of  the 
angles  of  the  mouth  being  drawn  upwards  or  outwards,  the 
upper  lip  is  elevated  and  the  mouth  forms  a  straight  line. 

The   shoulders   and   arms    are   affected  similarly  to   that 
already  described  under  the  Erb  or  juvenile  type. 


FAMILIAL  DISEASES  465 

7.  The  pelvic  type 

In  the  pelvic  type  (Buzzard)  the  paralysis  commences 
with  weakness  of  the  ilio-psoas  group,  so  that  a  difficulty  is 
experienced  in  going  up  stairs.  Owing,  however,  to  the  situa- 
tion of  the  muscles,  no  objective  examination  can  be  made. 
In  some  of  these  cases,  as  the  malady  progresses,  other 
muscles  of  the  pelvic  girdle  become  affected ;  in  other  cases, 
pseudo-hypertrophy  may  be  noted  in  some  of  the  muscles, 
such  as  the  infra- spinatus.  The  affection  as  a  rule  originates 
in  early  adult  or  later  life,  affects  females  more  commonly 


Fig.  155. — Illustrates  the  '  myopathic  fades  ' ;  observe 
the  straight  line  of  the  mouth  and  the  obliteration  of 
the  nasolabial  folds. 

than  males,  and  is  slowly  progressive,  so  that  the  fully 
developed  cases  present  the  features  already  described  under 
the  other  varieties. 

8.  The   distal  type 

In  the  distal  type  the  small  muscles  of  the  extremities 
are  primarily  affected.  In  this  type,  in  addition  to  the 
affection  of  the  small  muscles,  the  face  is  frequently  im- 
plicated. Although  this  presents  some  resemblance  to  the 
peroneal  type  of  muscular  atrophy,  the  two  conditions  are 
quite  distinct,  the  affection  of  the  face  and  the  absence  of 
sensory  changes  serving  to  distinguish  the  two  diseases. 

30 


i.;ii 


NERVOUS  DISEASES 


In  whatever  position  the  muscular  dystrophic  condition 
commences,  there  is  a  tendency,  as  the  malady  progresses,  for 
it  to  involve  other  muscular  groups,  so  that  eventually  the 
characteristic  attitude,  gait,  and  postures  already  described 
under  pseudo-hypertrophic  muscular  paralysis  are  assumed. 
On  the  other  hand,  cases  persist  for  a  number  of  years 
without  such  extension ;  in  these  instances  the  type  observed 
is  that  shown  in  the  earty  stages  only. 

Diagnosis.  The  chief  points  of  distinction  between 
muscular  dystrophies  and  the  atrophic  muscular  palsies  of 
nervous  origin  are  seen  in  the  following  table  : — 


Muscular  Dystrophy. 

Nkrvous  Amyotrophy. 

Age  at  onset     Childhood,  puberty, 
adolescence 

Adult  or  later  life 

Heredity 

Well  marked 

Usually  none 

Muscles 
aSected 

Facio-scapulo-humeral,      pelvic 
girdle,    &c,  i.e.   not   accord- 
ing   to  nuclear  grouping   in 
spinal  cord 

According  to  nuclear  group- 
ing and  arrangement  in 
spinal  segments 

Bulb 

No  bulbar  symptoms 

Frequently    bulbar    symp 
toms 

State  of 
muscles 

Pseudo-hypertrophy    of     some, 

atrophy  of  others 
Congenital      atrophy     or     ab 

sence  of  some  muscles 
No  fibrillary  twitchings 

Degenerative  atrophy   only 
occurs ;    no   hypertrophy 

Fibrillary  twitchings 

Reaction 

No  typical  R.D.,  but  reactions 
diminished  in    proportion  to 
atrophy 

In  acute  cases  the  R.D.  is 
well  marked 

Reflexes 
Sensation 

Tendon     reflexes      never     in 
creased.    Disappear  in  corre 
spondence  with  the  muscular 
affection 

Superficial  not  affected 

Never  affected 

Tendon  jerks  exaggerated ; 

ankle     clonus     may     be 

present 
Superficial  usually  lost 
Extensor  plantar  response 

Rarely  affected 

Sphincters 

Rarely  affected 

Rarely  affected 

From  the  atrophies  dependent  upon  lesions  of  the  peri- 
pheral nerves,  the  dystrophies  may  be  distinguished  by  the 
absence  of  sensory  symptoms  (objective  and  subjective)  and 
the  other  points  mentioned  in  the  above  table. 


FAMILIAL  DISEASES 


467 


Prognosis.  In  all  the  types  the  prognosis  as  regards 
recovery  is  bad.  The  pseudo-hypertrophic  variety  tends  to 
progress,  and  in  from  eight  to  ten  years  the  patient  is  unable 
to  stand  or  walk.  In  cases  which  arise  in  later  years  (the 
juvenile  forms)  the  progress  is  usually  slower  ;  and  in 
some  cases  the  affection 
remains  limited  to  the  parts 
originally  involved.  The  cause 
of  death  is  an  intercurrent 
affection,  especially  of  the 
respiratory  system,  such  as 
acute  pneumonia,  bronchitis, 
broncho-pneumonia,  or  pul- 
monary phthisis.  Any  ill- 
ness, which  obliges  the 
patient  to  take  to  bed,  is 
followed  by  a  rapid  increase 
of  paralysis,  and  patients 
able  to  walk  before  may  be 
unable  to  do  so  afterwards. 
Once  a  patient  is  unable  to 
get  about,  deformities  rapidly 
develop. 

Treatment.  These  patients 
should  be  encouraged  to  move 
about  and  use  the  muscles 
as  far  as  possible  short  of 
fatigue.  They  should  never  be  kept  in  bed  if  this  can  be 
avoided. 

Massage,  passive  movements,  and  faradism  help  to  main- 
tain and  improve  the  muscular  condition. 

General   tonic    treatment  should   be   prescribed,  and  care 
taken  to  prevent  respiratory  complications. 


Fig.  156  is  a  photograph  of  a  case  of 
Erb's  juvenile  muscular  atrophy. 


AMYOTONIA  CONGENITA 


This  is  a  rare  condition  characterised  by  a  congenital 
affection  of  the  muscles.  In  typical  cases  it  is  noticed  that 
shortly  after  birth  the  child's  limbs  are  unnaturally  flaccid 
and  hypotonic.     It  is  also  observed  that  sucking  is  impossible, 


30 


!<;s 


N'Ki; Vol's  DlSlvVSKS 


and  as  the  child  grows,  that  it  cannot  sit,  and  that  the  limbs 
may  assume  any  position  in  which  they  are  placed.  Later  on 
the  child  is  unable  to  walk,  the  movements  are  feeble,  the 
limbs  are  flail-like  at  a.ll  joints,  and  a  tendency  to  shorten- 


Fig.  157. 


Fig.  158. 

FlGS.  157  AND  158. — Illustrate  the  conditio)]  described  as 
amyotonia.  Owinp;  to  the  muscular  atonia  the  limbs  may  bo 
placed  in  various  abnormal  positions. 


ing  of  muscles  ensues,  which  may  limit  the  amount  of 
passive  movement.  The  electrical  reactions  are  quantita- 
tively diminished  and  the  deep  reflexes  are  abolished. 
There  are  no  sensory  changes.  The  mental  condition  is 
unaffected. 

The  tendency  of  the  malady  is  towards  improvement,  but 
as  none  of  the  cases  so  far  recorded  have  been  observed  over 
a  sufficient  period  of  time,  it  is  impossible  to  say  how  much 


FAMILIAL  DISEASES  409 

improvement  may  take  place,  or  what  may  be  the  duration 
of  life. 

In  two  cases  examined  pathologically,  changes  in  the 
muscles  and  in  the  anterior  roots  and  cornua  of  the  spinal 
cord,  similar  to  those  found  in  some  cases  of  muscular 
dystrophy,  have  been  described. 

It  is  impossible  to  exactly  classify  these  cases,  but  from 
the  point  of  view  of  morbid  histology  they  are  myopathic, 
although  clinically  distinct  from  the  muscular  dystrophies 
just  described. 


MYOTONIA  CONGENITA.     THOMSEN'S  DISEASE 

This  rare  condition  occurs  in  families  and  appears  to  be 
more  or  less  hereditary.  Its  onset  is  usually  in  early  child- 
hood. It  is  characterised  by  delayed  relaxation  of  the 
voluntary  muscles  after  contraction,  owing  to  the  presence 
of  tonic  spasm.  The  difficulty  may  exist  on  the  initial 
movement,  or  on  attempts  at  movement  after  a  period  of 
rest ;  but  after  repeated  efforts  the  spasm  becomes  pro- 
gressively less,  an  improvement  which  is  maintained  during 
continuance  of  the  effort. 

In  its  slighter  forms  the  disease  is  manifested  by  stiffness 
and  awkwardness  of  movement,  but  in  the  more  severe  forms 
it  may  completely  disable.  For  example,  the  patient  may  be 
unable  to  rise  from  bed  or  from  a  chair,  but  after  repeated 
efforts  the  spasm  becomes  less  and  finally  disappears,  so 
that  he  can  walk  for  miles  without  fatigue.  In  the  most 
severe  instances  the  spasm  may  affect  not  only  the  limbs, 
but  may  extend  over  the  whole  body,  rendering  it  rigid  and 
immobile. 

The  limbs,  especially  the  legs,  are  mainly  affected ;  respira- 
tion, micturition,  and  defecation  are  unaffected.  In  rare 
cases  mastication  and  swallowing  have  been  embarrassed. 

The  muscles  are  large  and  firm,  but  their  power  and 
strength  are  not  in  proportion  to  their  size.  They  possess 
an  increased  excitability  both  to  mechanical  and  electrical 
stimulation ;  and  the  contraction,  thus  excited,  persists  for 
a  considerable  period.  The  anodal  closing  contraction  is  as 
easily   obtained   as   the   cathodal.      A   continuous    galvanic 


170  NERVOUS  DISEASES 

current  causes  a  series  of  wave-like  contractions  from  the 
cathode  to  the  anode  (Erh). 

The  reflexes  are  unaltered. 

The  disease  persists  throughout  life  with  little  change. 
A  life  of  activity  tends,  on  the  whole,  towards  amelioration  of 
the  symptoms. 

The  condition  is  characterised  pathologically  by  a  hyper- 
trophy of  the  muscular  fibres  and  a  proliferation  of  their 
nuclei. 

MYOTONIA  ATROPHICA 

This  is  a  very  rare  condition,  which  never  arises  before 
puberty.  It  is  characterised  by  a  myotonic  state  of  the 
muscles  of  the  extremities,  especially  of  the  hands.  Owing  to 
this  the  patient  is  unable  to  relax  his  grasp  suddenly.  In 
addition  to  the  muscular  tonicity,  atrophy  may  be  present. 
The  muscles  usually  affected  are  :  the  facial  muscles,  the 
sterno-mastoids,  vasti  femoris,  and  dorsiflexors  of  the  feet. 

FAMILY  PERIODIC  PARALYSIS 

This  is  a  rare  disease,  characterised  by  recurring  attacks 
of  paralysis  of  the  voluntary  muscles,  with  temporary  loss  of 
electrical  excitability. 

It  is  essentially  a  faruity  disorder,  and  has  in  one  instance 
been  traced  through  five  generations.  It  is  commonest  in 
children  and  young  adults.  Fatigue  would  appear  to  be  its 
chief  exciting  cause. 

Pathology.  The  disease  appears  to  be  of  a  toxic  nature. 
Slight  degrees  of  lymphocytosis  have  been  observed  on 
examination  of  the  blood,  and  portions  of  muscles  excised 
during  life  have  shown  marked  vacuolation  and  Assuring.  A 
hyper  toxicity  of  the  urine  has  been  observed  after  an  attack 
of  paralysis.  It  has  been  suggested  by  Buzzard  that  the 
action  of  the  toxic  substance  is  upon  the  muscular  rather  than 
upon  the  nervous  tissues. 

There  is  so  far  no  record  of  any  post-mortem  examination. 

Symptoms.  The  attacks  of  paralysis  follow  upon  physical 
exertion,  some  hours  intervening  between  exercise  and  the 
onset  of  the  paralytic  symptoms. 

The   attacks  consist  of  motor  paralysis  of  a  flaccid  type, 


FAMILIAL  DISEASES  17] 

commencing  in  the  proximal  muscle.-  of  the  limbs  and  then 
invading  the  whole  limb,  the  muscles  of  the  trunk,  and  the 
respiratory  muscles.  The  diaphragm  is  rarely  affected,  but 
the  area  of  cardiac  dullness  is  increased  and  a  systolic  bruit 
may  dev  Lop  ~  the  mitral  orifice.  The  cranial  nerves  are 
rarely  paralysed,  although  ptosis  and  bulbar  symptoms  have 
been  occasionally  observed. 

The  palsy  is  of  the  flaccid  type,  the  patient  lying  helpless 
in  bed.  br-athin_-  -Yy  with  difficulty.  The  deep  reflexes  are 
in  abeyance,  and  no  response  is  obtained  to  either  galvanic  or 
faradic  stimulation.     This  is  a  characteristic  feature. 

The  duration  of  the  attacks  varies  from  six  or  eight  to 
sixty  or  more  hours.  Their  disappearance,  like  then-  onset,  is 
gradual.  The  intervals  between  the  attacks  also  vary  from 
a  few  hours  to  several  months.  There  would  seem  to  be  a 
tendency  hi  the  course  of  time  for  the  intervals  to  lengthen 
and  the  attacks  to  shorten. 

Prognosis  The  outlook  as  regards  life  is  favourable.  No 
patient  so  far  has  died  of  the  disease. 

Treatment  The  most  successful  procedure  has  been  the 
administration  of  diuretics  and  copious  libations  of  soda 
water.  The  elimination  of  toxic  substances  is  in  this  way, 
encouraged.  Tonic  treatment,  moderate  exercise,  and  simple 
easily  digested  food  may  also  be  prescribed  with  advantage. 

THE    FAMILY  FORM    OF    MUSCULAR  ATROPHY  OF 
SPINAL  TYPE  IY  CHILDREN  AYERDYIG-HOFFMAYhY 

This  disease  commences  during  the  latter  half  of  the  first 
year  of  life,  and  is  characterised  by  the  development  of  weak- 
ness and  atrophy  of  the  muscles.  It  occurs  in  more  than  one 
member  of  the  family,  and  runs  a  similar  course  in  each  case 
The  weakness  is  first  seen  in  the  muscles  of  the  back  and 
hips,  and  gradually  spreads  to  involve  the  limbs,  including  the 
small  muscles  of  the  hands.  Later,  bulbar  weakness  is 
noticed.  The  onset  of  the  paralysis  is  followed  by  a  trophy } 
more  or  less  rapid  and  extensive,  with  occasionally  fibrillary 
tremor.  The  reaction  of  degeneration  is  present.  Sensation 
is  unimpaired,  the  sphincters  are  normal,  and  the  deep  reflexes 
are  lost. 

As  the  malady  progresses,  the  child  becomes  helpless  and 


17L1 


NERVOUS  DISEASES 


paralysed.     Death  frequently  results  from  respiratory  com- 
plications, but  may  not  take  place  for  three  or  four  years. 

Pathology.  Atrophy  of  the  muscular  fibres  is  associated 
with  atrophy  of  the  nerves,  anterior  roots,  and  anterior  horn 
cells  of  the  cord. 


PERONEAL  MUSCULAR  ATROPHY 

This  disease  commences  between  the  ages  of  fifteen    and 
forty  years,  but    usually  before  twenty-five.      One    or   more 

members  of  a  family 
are  affected,  the  males 
more  commonly  than 
the  females.  It  may 
be  directly  hereditary, 
or  may  miss  a  genera- 
tion. 

Pathology.  The 
changes  found  in  the 
nervous  system  consist 
of  muscular  atrophy  of 
neuritic  type,  degen- 
eration of  the  mixed 
nerves,  of  the  anterior 
nerve  roots  and 
anterior  horn  cells, 
and  also  in  lesser 
degree  of  the  posterior 
roots  and  posterior 
columns.  It  is  there- 
fore quite  distinct  from 
the  myopathies,  and 
must  be  placed 
amongst  the  muscular 
atrophies  of  nervous 
origin. 
Symptoms.  It 
runs  a  slow  course,  extending  over  many  years.  It  is 
characterised  by  the  development  of  atrophy  of  the  small 
muscles  of  the  feet,  the  peronei  muscles,  the  extensor  hallucis, 
extensor  communis   digitorum,    and   later  the  gastrocnemii. 


Fig.  159. — Illustrates  the  wasting  of  the  ontei 
aspect  of  tin-  i-itrlit  Leg  in  a  case  of  right- 
sided  peroneal  muscular  atrophy. 


FAMILIAL  DISK  AS  US 


473 


A  similar  condition  affecting  the  small  hand  muscles  and 
those  of  the  thenar  and  hypothenar  groups,  with  con- 
sequent deformity,  develops  later  in  the  upper  limbs.  The 
appearance  of  the  leg  is  typical.  In  the  majority  of  cases 
the  foot  is  lengthened  and  inverted,  the  toes  pointed,  the 
leg  below  the  knee  wasted,  especially  on  the  peroneal  aspect. 
In  some  cases  the  ankle  joint  is  loose  and  flaccid,  in  others 
it  is  ankylosed.  Deformities  in  the  shape  of  talipes  varus 
and  equino  varus  are  sometimes  present.  Fibrillary  twitch- 
ings  and  an  incomplete  reaction  of 
degeneration  are  seen  in  the  muscles. 

Impaired  electrical  irritability  to 
faradism  alone,  or  in  association 
with  muscular  atrophy,  may  be 
observed  over  a  wider  distribution. 

The  ability  displayed  in  walking 
is  surprising,  considering  the  atrophic 
state  of  the  limbs  and  weakness  of 
the  muscles. 

Sensory  symptoms  such  as  pain 
are  occasionally  present,  and  slight 
but  definite  disturbance  of  sensibility 
may  be  found  on  the  outer  side  of 
the  leg.  There  is  no  tenderness  on 
pressure  along  the  nerves. 

The   deep   reflexes    diminish     and 
disappear   according  to   the  muscles 
affected.     The    superficial   remain   normal 
are  unaffected. 

Course  and  prognosis.  Life  is  not  shortened  by  the 
disease,  which  may  run  a  course  of  many  years.  A  certain 
number  of  cases  appear  to  become  arrested,  though  the  greater 
number  develop  atrophy  in  the  distal  segments  of  the  lower 
and  upper  limbs. 

The  treatment  is  conducted  along  the  lines  for  atrophic 
palsies  in  general.  Orthopaedic  appliances,  or  surgical  mea- 
sures, may  be  adopted  for  individual  cases  with  great 
benefit. 


Fig.  160. — Appearance  of 
the  legs  and  feet  in  a 
case  of  peroneal  muscular 
atrophy. 


The   sphincters 


474  NERVOUS  DISEASES 


FRIEDREICH'S   DISEASE 

This  is  a  progressive  malady,  often  occurring  in  families, 
characterised  clinically  by  the  gradual  onset  of  inco-ordination 
of  movement,  impairment  of  articulation,  and  the  development 
of  deformities  of  the  back,  feet,  and  hands.  The  pathological 
changes  consist  of  a  system-degeneration  of  the  spino- cerebellar 
tracts,  and  of  sclerosis  of  the  posterior  and  lateral  columns  of 
the  spinal  cord. 

Etiology.  It  commences  during  the  early  years  of  life, 
most  commonly  between  the  seventh  and  seventeenth  years. 
It  affects  males  rather  more  often  than  females.  It  can 
scarcely  be  called  an  hereditary  disease,  as  it  is  rarely  seen  in 
parent  and  offspring.  It  is  more  strictly  a  familial  disorder, 
affecting  several  members  of  the  same  family.  It  would 
therefore  appear  as  if  there  was  a  congenital  tendency  rather 
than  a  direct  inheritance  to  the  malady.  Consanguinity, 
alcoholism,  and  syphilis  have  been  noted  in  the  parents  in 
several  cases ;  but  no  constant  causal  factor  has  been 
ascertained.  It  is  rare  to  find  any  exciting  cause  for  the 
disease. 

Pathological  anatomy.  Degeneration  is  found  in  the 
posterior  columns,  the  spino-cerebellar,  and  the  pyramidal 
tracts.  Both  sides  of  the  cord  are  symmetrically  degenerated, 
and  the  dorsal  region  is  often  more  affected  than  the  other 
portions. 

In  the  posterior  columns,  the  postero-internal  tract  is  more 
sclerosed  than  the  posterior  root  zone.  The  cells  and  fibre 
network  of  Clarke's  column  are  degenerated,  a  condition  which 
is  associated  with  sclerosis  of  the  direct  cerebellar  tract  and  of 
Gowers's  tract,  the  latter  in  more  advanced  cases. 

The  cerebellum  only  exceptionally  shows  atrophy  of  the 
Purkinje"  cells,  probably  secondary  to  sclerosis  of  the  spino- 
cerebellar tracts. 

Three  explanations  have  been  given  to  account  for  the 
changes  observed  in  the  spinal  cord  in  this  disease.  First,  a 
tendency  for  degenerative  changes  to  occur  in  the  peripheral 
parts  of  the  cord,  which  are  less  freely  supplied  with  blood 
than  the  central  areas  (Williamson).  Secondly,  an  inherited 
tendency  towards  general  early  vascular  deterioration  (Pitt). 


FAMILIAL  DISEASES  475 

Thirdly,  abiotrophy,  or  an  inherent  tendency  towards  early 
death  of  the  nerve  fibres  (Gowers). 

In  view  of  the  pathological  findings  in  some  of  the  allied 
disorders  about  to  be  described,  we  are  of  opinion  that  the  last 
of  these  theories  affords  the  most  probable  explanation. 

Symptoms.  The  disease  commences  slowly  and  gradually, 
with  unsteadiness  and  inco-ordination,  resulting  in  an  instability 
both  in  standing  and  walking,  which  may  bring  about  frequent 
falls,  and  oblige  the  child  to  abstain  from  playing  with  other 


Fig.  161. — Illustrates  the  characteristic  appearance  of  the  feet  in 
Friedreich's  disease. 


children.  The  gait  is  irregular,  the  feet  are  set  far  apart,  and 
the  movements  are  clumsy  and  inco-ordinate.  As  time  goes 
on,  the  patient  may  require  the  aid  of  sticks  or  crutches,  but 
gradually  inco-ordination  affects  the  muscles  of  the  arms  and 
trunk,  so  that  eventually  walking  becomes  impossible.  In 
this  stage  tremor  and  unsteadiness  of  the  trunk,  arms,  and 
head  are  observed,  and  as  a  rule  definite  and  characteristic 
deformities  of  the  back  and  feet. 

The  deformity  first  seen  is  pes  cavus,  a  condition  in  which 
the  foot  is  slightly  dropped  and  foreshortened,  the  heel  drawn 
up,  the  dorsum  arched,  the  sole  hollowed  out,  the  toes  hyper- 
extended  at  the  metatarso-phalangeal,  but  flexed  at  the 
inter-phalangeal  joints.  Over-extension  of  the  great  toe  is 
especially  striking.     The  digital  end  of  the   foot  is  square. 


47li 


NERVOUS  DISEASES 


When  looked  at  in  profile  from  the  inner  aspect,  the  foot 
presents  a  rough  Z-shaped  appearance.  This  deformity  may 
be  present  before  any  considerable  degree  of  unsteadiness  or 
inco-ordination  is  noted.     (Fig.  161.) 

The  spinal  deformity  which  is  characteristic  of  the  ad- 
vanced cases  develops  later,  and  consists  of  lateral  curvature 
(scoliosis)  with  occasionally  kypho-scoliosis.  (Fig.  162.)  In 
the  hands  minor  degrees  of  the  '  main  en  griffe '  may  be 
observed,  but  this  is  relatively  rare. 

The  speech  is  characteristic,  and  is  of  a  hesitating,  syllabic, 
explosive  order.  It  results  from  inco- 
ordination of  the  articulatory  and  re- 
spiratory muscular  mechanisms.  On 
attempting  to  speak,  the  expiratory  and 
facial  muscles  are  thrown  into  strong 
contraction,  but  the  glottic  opening 
remains  closed.  After  an  effort  the  air 
passes  through  the  glottis,  giving  vent 
to  a  loud  explosive  sound. 

Mental  condition.  Some  degree  of 
mental  dullness  is  usually  observed, 
not  amounting  to  mental  deficiency, 
but  rather  to  a  want  of  mental  acute- 
ness  and  activity.  The  disposition  is 
happy  and  placid,  often  approaching 
childishness. 

Cranial  nerves.  Smell,  taste,  and 
hearing  are  not  affected.  Vision  may  be 
impaired.  Although  optic  atrophy  was 
stated  never  to  occur,  several  cases  are 
now  on  record  hi  which  it  has  been 
observed ;  its  occurrence,  however,  is 
rare.  There  is  no  paralysis  of  the 
ocular  muscles,  but  a  weakness  of  sustained  conjugate  move- 
ment is  occasionally  found.  Nystagmus  is  a  common 
symptom  occurring  in  the  form  of  slow  nystagmoid  jerkings 
of  the  globes  in  all  directions.  The  pupils  are  normal 
in  size,  shape,  and  reaction,  but  in  a  few  cases  inequality  and 
impaired  light  reaction  have  been  observed  in  association 
with  changes  characteristic  of  congenital  syphilis. 

The  bulbar  nerves  are  not  paralysed,  but  inco-ordination  of 


Fig.  162.— Photograph 
of  a  case  of  Fried- 
reich's disease  slmw  ing 
the  spinal  curvature 
(scoliosis). 


FAMILIAL  DISEASES  477 

the   muscular   movements  gives  rise   to  a  peculiar  form  of 
articulation,  which  has  been  already  described  (p.  476). 

Motor  system.  At  first  there  is  no  loss  of  muscular  power, 
but  in  the  later  stages  a  gradual  and  obvious  impairment 
develops,  which  becomes  marked  in  the  terminal  stages.  It  is 
probably  owing  to  the  motor  weakness  that  the  deformities 
of  the  feet,  spine,  and  hands  are  brought  about.  Slight 
general  muscular  wasting  is  found,  but  .there  is  no  atrophy, 
fibrillation,  or  electrical  changes  characteristic  of  muscular 
degeneration.  Hypotonia  is  usually  present,  especially  in 
the  early  stages,  but  during  the  later  spasticity  and  con- 
tractures may  supervene. 

Sensory  system.  In  a  certain  number  of  cases  complaint 
is  made  of  cramp-like  or  darting  and  dull  aching  pains, 
but  these  are  never  severe.  In  most  cases  no  objective 
loss  of  sensation  is  detected ;  loss  of  the  sense  of  posi- 
tion is  quite  exceptional,  but  slight  impairment  of  both  the 
superficial  and  deep  forms  of  sensibility  may  sometimes  be 
detected. 

Beflexes.  The  deep  reflexes  may  at  first  be  unaffected, 
but  later  become  diminished  and  eventually  abolished.  The 
plantar  reflex  is  extensor  in  type,  and  the  superficial  epi- 
gastric and  abdominal  reflexes  are  usually  lost. 

The  sphincters  are  rarely  affected.  Trophic  changes, 
though  not  unknown,  are  uncommon.  Visceral  crises  are 
never  present. 

An  abnormal  type  of  the  disease  is  seen  in  cases  which 
commence  with  symptoms  of  ataxic  paraplegia,  nystagmus, 
articulatory  defects,  increase  of  the  deep  reflexes,  extensor 
responses,  and  sphincter  trouble.  When  seen  in  the  early 
stage,  these  cases  present  a  typical  picture  of  disseminated 
sclerosis,  but  occurring  at  an  age  when  disseminated  sclerosis 
is  rare  or  unknown.  In  two  such  cases,  the  subsequent  loss 
of  the  deep  reflexes  and  the  development  of  characteristic 
deformities  changed  the  picture  of  disseminated  sclerosis  into 
one  of  Friedreich's  disease. 

The  differential  diagnosis  has  to  be  made  from  juvenile 
tabes  (p.  399),  disseminated  sclerosis  (p.  418),  Pott's  disease 
(p.  357),  and  cerebellar  tumour  (p.  262). 

The  course  of  the  disease  is  chronic,  the  symptoms  lasting 
for  thirty  or  more  years.     Death  results  from  intercurrent 


478  NERVOUS  DISEASES 

affections,  not  infrequently  from  cardiac  failure,  independent 
of  valvular  disease. 

The  treatment  is  limited  to  the  alleviation  of  symptoms 
as  they  arise,  in  association  with  massage,  passive  movements, 
and  Frsenkel's  exercises  (p.  399). 

CHRONIC  DISORDERS  GIVING  RISE  TO  SYMPTOMS 
OF  CEREBELLAR   DISEASE 

Under  the  title  of  Hereditary  Cerebellar  Ataxia,  Marie 
described  a  series  of  cases,  collected  from  the  literature,  in 
which  signs  of  cerebellar  disease  occurred,  sometimes  in 
families  and  usually  starting  in  early  adult  life.  As  Holmes  l 
has  shown,  the  anatomical  investigation  and  the  clinical  study 
of  the  cases  do  not  justify  the  application  of  this  term,  and 
he  states  that  no  form  of  disease  exists  to  which  this  desig- 
nation may  be  fitly  given.  The  following  classification, 
suggested  by  Holmes,  would  appear  to  be  at  once  the  most 
natural  and  the  simplest  wherewith  to  study  these  disorders. 

The  cases  fall  into  the  following  four  subdivisions  :  — 

1.  Primary  parenchymatous  degeneration  of  the  cere- 
bellum. 

2.  Olivo-ponto-cerebellar  atrophy. 

3.  Degeneration  of  the  spinocerebellar  tracts. 

4.  Congenital  smallness  of  the  central  nervous  system, 
associated  with  cerebellar  symptoms. 

These  disorders  are  all  characterised  by  a  symptomatology, 
which  does  not  differ  materially  in  its  main  features  in  any 
of  the  particular  varieties. 

The  general  symptoms  may  be  stated  to  be 

(a)  A  reeling,  staggering,  or  drunken  gait  characteristic  of 
cerebellar  disorders  ;  (b)  irregularity,  uncertainty,  and  some- 
times tremor  in  the  movements  of  the  arms ;  and  (c)  a 
hesitating,  scanning,  and  usually  explosive  form  of  articulation. 
Certain  individual  peculiarities  have  also  been  observed,  to 
which  brief  attention  will  presently  be  directed. 

The  underlying  pathological  changes   are    in    some  cases 
degeneration   or  sclerosis   of  the  cortex   of   the    cerebellum. 
In  other  cases  the  sclerotic  change  is  confined  to  some  portion 
of  the  afferent  or  efferent  cerebellar  systems. 
1  Holmes,  Brain,  11)07 


FAMILIAL  DISEASES  479 

r.  Primary  progressive  cerebellar  degeneration  (Holmes) 

This  is  a  familial  disorder,  occurring  in  adults  between 
the  ages  of  thirty  and  forty,  and  progressing  slowly  to  a  fatal 
termination  after  a  number  of  years.  Cases  of  a  somewhat 
similar  type  have  also  been  described  by  Fraser l  as  occurring 
in  children.  Its  primary  and  outstanding  features  are  :  (1)  a 
reeling  or  staggering  gait ;  (2)  inco-ordination  and  uncertainty 
in  the  movements  of  the  arms ;  (3)  a  hesitating,  scanning, 
and  explosive  articulation ;  and  (4)  tremors  of  the  head  and 
limbs  and  nystagmus.  No  motor  or  sensory  paralysis  has 
been  observed,  nor  is  there  any  organic  change  in  the 
reflexes.     The  mental  condition  is  unaffected. 

Microscopical  examination  reveals  a  primary  and  pro- 
gressive degeneration  of  the  cortex  of  the  cerebellum,  shown 
mainly  in  a  disappearance  of  the  cells  of  Purkinje  and  their 
efferent  fibres.  A  secondary  neuroglial  proliferation  and 
sclerosis  are  found  in  the  subcortical  cerebellar  white 
matter.  The  spino-cerebellar  and  efferent  cerebellar  tracts 
are  intact. 

2.  Olivo-ponto-cerebellar  atrophy  (Thomas) 

This  condition  is  neither  hereditary,  familial,  nor  congenital. 
It  commences  at  a  late  period  of  life  and  progresses  to  a  fatal 
termination.  It  is  characterised  clinically  by  defective  equili- 
bration in  standing  and  walking,  inco-ordination  and  tremor 
of  the  arms  may  or  may  not  be  present,  articulation  is  slow 
and  scanning,  and  nystagmus  is  usually  observed. 

The  pathological  lesions  are  atrophy  of  the  cerebellar  cortex, 
degeneration  of  the  inferior  olivary  bodies,  and  atrophy 
of  the  grey  substance  or  nuclei  of  the  pons.  The  middle 
cerebellar  peduncles  are  completely,  and  the  restiform  bodies 
partly,  degenerated. 

In  other  cases,  which  seem  to  form  a  connecting  link 
between  Friedreich's  disease  and  that  next  to  be  described, 
Thomas  and  Menzel  found,  in  addition  to  the  changes  just 
recorded,  an  atrophy  of  the  spino-cerebellar  tracts,  with  which 
was  associated  some  degree  of  degeneration  of  the  posterior 

1  Fraser,  Glasgoiv  Medical  Journal,  1880. 


480  NERVOUS  DISEASES 

and  lateral  columns  of  the  spinal  cord.  This  type  was  seen 
in  younger  patients,  was  a  familial  disorder  and  presented,  in 
addition  to  the  cerebellar  symptoms,  spasticity  of  the  legs 
and  increase  of  the  deep  reflexes. 

3.  Degeneration  of  the  spino-cerebellar  tracts 
(Klippel  and  Durante  ;  Sanger  Brown) 

This  is  a  familial  condition,  coming  on  between  the  ages 
of  sixteen  and  thirty-five.  It  is  characterised  by  a  reeling 
cerebellar  gait,  without  any  tendency  to  Eombergism,  ataxia 
of  the  legs,  arms,  facial  and  ocular  muscles,  nystagmus,  and 
indistinct  and  scanning  articulation.  Sometimes  failure  of 
vision  from  optic  atrophy  is  observed.  There  may  or  may 
not  be  interference  with  the  pupillary  light-reflex.  Rigidity, 
weakness  of  voluntary  movements  of  the  limbs,  and  con- 
tractures of  the  legs  are  seen  in  the  later  stages ;  but,  except 
for  the  contractures,  no  deformities  are  present.  The  tendon 
reflexes  are  normal  or  exaggerated. 

The  outstanding  pathological  feature  in  these  cases,  of 
which  quite  a  number  have  been  examined,  was  observed  to 
be  a  complete  degeneration  of  the  dorso-cerebellar  tract  of 
Flechsig,  with  degeneration  and  atrophy  of  the  cells  of  Clarke's 
column.  The  ventro-cerebellar,  or  tract  of  Gowers,  was  also 
affected,  but  to  a  less  extent.  The  posterior  columns  of  the 
cord  were  in  part  degenerated.  The  nervous  system  generally 
was  small,  but  very  little  alteration  was  observed  in  the 
cortex  of  the  cerebellum. 

4.  Congenital  smallness  of  the  central  nervous  system 
with  cerebellar  symptoms  (Nonne) 

This  is  probably  a  developmental  condition,  and  the 
symptoms  arise  during  puberty  or  adolescence.  These  are 
uncertain  gait,  explosive  articulation,  nystagmoid  jerkings  of 
the  eyes,  and  optic  atrophy.  The  mental  condition  may 
also  show  deterioration.  The  whole  central  nervous  system, 
especially  the  cerebellum,  is  unusually  small.  No  definite 
degeneration  of  the  nervous  system  has  been  detected. 


FAMILIAL  IMS HANKS 


48! 


HEREDITARY    [FAMILIAL]    SPASTIC    PARAPLEGIA 

This  disorder  is  characterised  by  the  gradual  onset  of 
spastic  paralysis,  which  commences  in  early  life  from  seven 
or  eight  years  onwards.  In  some  cases  it  is  hereditary,  but 
more  commonly  familial,  while  not  a  few  instances  of  sporadic 
occurrence  have  been  recorded. 


Ki.,.  11;:;. 


Fig.  164. 


Figs.  163  and    164. — Illustrate    spastic    contractures    in    a    case    of    family 
spastic  paralysis. 


Spastic  paralysis  affecting  several  members  of  one  family 
may  be  due  to  an  individual  cause  affecting  all  the  children  of 
certain  parents,  without  being  in  any  degree  truly  hereditary. 

The  morbid  changes  consist  of  degeneration  in  the  crossed 
pyramidal  tracts,  and  sometimes  also  of  the  postero-internal 
column. 

Symptoms.  In  its  pure  form  the  disease  is  characterised 
by  the  development  of  a  spastic  paraplegia,  chiefly  affecting  the 
lower  limbs  and  sometimes  the  upper,  without  involvement  of 

31 


4S2  NERVOUS  DISEASES 

the  face.  The  deep  reflexes  are  increased,  extensor  plantar 
response  is  present,  and  in  some  cases  slight  sphincter 
trouble.  The  mental  condition  is  good.  Ocular  symptoms 
are  not  present.  There  is  neither  inco-ordination  nor  loss  of 
sensation. 

The  malady  is  progressive,  increasing  to  complete  paralysis 
with  spasticity,  adductor  spasm,  and  drawing  up  of  the  heels. 

Its  distinction  from  cerebral  diplegia  is  based  upon  the 
later  onset  of  the  paralysis,  the  absence  of  mental  enfeeble- 
ment,  and  the  slowly  progressive  character  of  the  symptoms. 

In  a  second  type,  in  addition  to  the  spastic  symptoms, 
deformities  of  the  feet,  instability,  and  slight  degrees  of 
inco-ordination  have  been  described.  Cases  have  also  been 
recorded  in  which  wasting  of  the  small  muscles  of  the  hand 
has  been  observed. 

There  are  on  record  cases  of  family  disease  in  which  the 
clinical  picture  has  shown  every  grade  of  variation,  from  a 
purely  spastic  type  to  the  well-marked  '  complex '  of  Fried- 
reich's disease  and  '  hereditary  cerebellar  ataxy.'  The  class 
into  which  many  of  the  cases  may  be  placed  varies  with  the 
progress  of  the  malady. 

Although  certain  definite  types  may  therefore  be  recog- 
nised, it  is  only  the  post-mortem  examination  which  can 
decide  the  final  classification. 

AMAUROTIC  FAMILY  IDIOCY 

This  is  a  rare  disease,  characterised  by  the  gradual  onset 
of  blindness,  with  mental  and  physical  impairment,  and 
ending  invariably  in  death. 

It  is  practically  confined  to  children  of  Jewish  parentage, 
and  may  occur  in  more  than  one  member  of  the  same  family. 
It  is  characterised  by  disappearance  of  the  cortical  cells,  and 
by  some  degree  of  degeneration  of  the  myeline  sheaths  of  the 
cortico-spinal  efferent  system. 

The  child  is  apparently  healthy  at  birth,  but  at  or  about 
four  months  of  age  paralytic  weakness  appears  in  the 
muscles  of  the  neck,  body,  and  limbs.  At  first  the  palsy  is  of 
jtbe  flaccid  type,  but  soon  some  degree  of  spasticity  makes  its 
appearance  with  wasting,  more  especially  of  the  muscles  of 
the  hands.     Concurrently  with  the  motor  symptoms,  a  change 


FAMILIAL  DISEASES  483 

in  the  mental  state  of  the  child  is  noted  by  the  mother.  It 
ceases  to  take  interest  in  its  surroundings,  becomes  apathetic, 
and  shows  signs  that  vision  is  also  impaired.  This  progresses 
to  complete  blindness ;  and  with  the  increase  in  the  loss  of 
vision,  the  muscular  paralysis  becomes  more  definite  and 
spastic.     The  reflexes  remain  unaffected. 

The  ocular  phenomena  are  characteristic  of  the  malady. 
At  the  macula  a  cherry-red  spot  is  seen,  surrounded  by  a 
white  halo.  There  is  atrophy  of  the  optic  discs,  and  vision  is 
impaired  or  altogether  abolished.  The  pupillary  light  reaction 
is  defective,  and  nystagmus  may  appear  later. 

The  malady  runs  a  course  of  about  two  years,  and  in- 
variably terminates  fatally. 

No  treatment  has  proved  of  any  avail  in  arresting  the 
disease.  It  does  not  appear  to  have  any  relation  to  congenital 
syphilis. 

Its  nature  is  still  a  subject  of  discussion.  Four  theories 
have  been  advanced  to  account  for  it :  (1)  cortical  agenesis 
(Sachs) ;  (2)  primary  cortical  degeneration  (Russell  and 
Kingdom)  ;  (3)  a  toxic  degeneration  of  the  motor  neurones 
(Hirsch) ;  and  (4)  degeneration  resulting  from  an  inherent 
bio-chemical  property  of  the  protoplasm  of  the  nerve  cells 
(Holmes). 

HEREDITARY  CHOREA.     HUNTINGTON'S  CHOREA 

This  is  a  rare  disease,  superficially  resembling  chorea  minor, 
but  occurring  in  adults  in  whose  ancestry  it  has  also  been 
found.  It  is  characterised  by  the  presence  of  involuntary, 
purposeless  movements  in  conjunction  with  psychical  symp- 
toms, which  progress  towards  dementia. 

According  to  Huntington,  three  features  characterise  the 
disease  :  its  hereditary  nature,  its  manifestation  in  adult  life, 
and  a  tendency  towards  mental  impairment  and  suicide. 

Etiology.  The  existence  of  the  disease  in  one  or  other 
parent  is  constant.  Should  it,  however,  miss  a  generation,  it 
is  unlikely  that  it  will  reappear  in  succeeding  generations. 
Several  members  of  the  same  family  and  generation  may  be 
subject  to  it.  The  most  common  age  for  its  appearance  is 
between  thirty  and  forty.  It  never  commences  before  thirty, 
but  may  develop  after  forty. 

31  * 


L84  NERVOUS  DISEASES 

Symptoms.  The  motor  symptoms  resemble  those  seen 
in  chorea  minor.  They  are  of  an  involuntary,  purposeless 
character,  affecting  different  groups  of  muscles,  but  rarely 
presenting  the  quick,  jerking  character  of  the  movements  of 
true  chorea,  and  often  distinctly  inco-ordinate  rather  than 
choreiform  (Osier).  They  cease  during  sleep  and  are  accen- 
tuated by  excitement. 

In  the  face  grimaces  and  gesticulations  are  common.  The 
speech  is  slow  and  hesitating,  and  eventually  becomes  indis- 
tinct. The  gait  is  erratic,  the  patient  for  instance  suddenly 
stopping  and  resuming  after  a  short  interval.  On  the  other 
hand,  it  may  be  swaying  and  unsteady,  the  feet  being  set 
far  apart  and  the  arms  in  constant  motion. 

The  psychical  features  are,  in  the  early  stages,  irritability 
and  depression,  often  leading  to  suicide ;  and  in  the  later, 
definite  mental  enfeeblement  progressing  to  dementia. 

The  duration  of  the  disease  is  prolonged,  sometimes  to 
thirty  years.  It  is  incurable,  the  tendency  being  towards  loss 
of  motor  power,  suicide,  or  dementia. 


Part  XII 

DISEASES   CHARACTERISED  BY  DISORDERS 
OF   MUSCULAR   FUNCTION 

MYASTHENIA    GRAVIS 

This  is  a  subacute  or  chronic  disease,  characterised 
clinically  by  the  rapid  development  of  muscular  fatigue  and 
weakness  on  exertion.  It  affects  most  commonly  the  bulbar, 
facial,  and  external  ocular  muscles,  but  may  be  general  in 
distribution. 

Etiology.  It  occurs  about  equally  in  the  sexes,  with,  if 
anything,  a  preponderance  in  the  female  sex,  attacking  women 
at  a  somewhat  earlier  age  than  men.  It  may  come  on  at  any 
time  during  adolescent  or  adult  life.  The  malady  is  neither 
hereditary  nor  familial. 

Pathology.  Changes  in  the  nervous  system  are  neither 
constant  nor  striking.  In  one  case,  described  by  Buzzard,1 
small  collections  of  lymphocytes  were  observed  scattered 
between  the  nerve  cells  of  the  bulbar  nuclei— these  were 
similar  to  the  '  lymphorrhages '  seen  in  other  tissues— and  in 
another  case  a  similar  condition  was  observed. 

The  most  prominent  change  is  the  presence  of  widely 
distributed  cellular  and  sometimes  serous  exudations  (lym- 
phorrhages) in  the  tissues  and  organs  of  the  body,  especially  of 
the  muscles,  liver,  and  kidneys.  In  the  muscles,  slight  fibre 
alterations,  suggestive  of  toxic  degeneration,  are  frequently 
present.     Pronounced  muscular  atrophy  is  rare. 

Proliferative  and  degenerative  changes,  and  new  growths 
in  the  thymus  gland,  although  not  constant,  have  been 
observed  in  a  number  of  cases. 

1  Farquhar  Buzzard,  Brain,  1905. 
485 


486  NERVOUS  DISEASES 

The  most  likely  explanation  is  that  the  disease  is  due  to 
the  presence  of  a  toxic  or  autotoxic  agent,  which  has  a 
special  effect  upon  the  protoplasmic  constituents  of  the 
voluntary  muscles.  It  is  possible  that  the  '  lyruphoiThages,' 
which  have  been  frequently  observed,  are  an  expression  of 
the  action  of  this  toxic  agent.  The  role  played  by  the  thymus 
gland  in  the  production  of  the  disease  is  not  clear. 

Symptoms.  The  symptoms  presented  by  this  disease 
vary  considerably,  not  only  in  different  cases,  but  in  each 
individual  case.  There  are,  however,  certain  features  which 
remain  common  to  all  cases,  namely,  weakness  of  the  facial 
and  external  ocular  muscles,  with  or  without  weakness  of  the 
palatal  and  articulatory  muscles.  This  weakness  is  increased 
or  brought  on  by  fatigue,  and,  as  a  rule,  is  worse  at  the  end 
of  the  day.  It  may  give  rise  to  slurred  speech,  impair- 
ment of  mastication  and  swallowing,  and  in  some  cases  to 
diplopia. 

These  symptoms,  at  first  transient,  may  be  ascribed  to 
hysteria  in  the  absence  of  definite  weakness,  but  every  case 
should  be  examined,  and  if  this  is  done  and  an  electrical 
examination  of  the  muscles  made,  the  true  nature  of  the  con- 
dition will  be  at  once  revealed.  There  is  often  superadded 
a  general  weakness  and  feeling  of  fatigue  and  tiredness,  with 
heaviness  of  the  limbs,  and  sometimes  dull  aching  pains. 

In  any  particular  case  the  local  incidence  of  weakness  may 
be  determined  by  the  occupation,  which  may  necessitate  the 
employment  of  one  group  of  muscles  more  than  another.  For 
example,  a  painter  whose  work  necessitated  his  looking  up  and 
using  his  right  arm,  developed  weakness  of  the  ocular  and 
retrocollic  muscles,  and  of  the  right  deltoid  muscle.  A  teacher 
who  had  to  talk  during  the  day  found  that  towards  evening 
she  could  hardly  make  herself  understood,  her  tongue  felt  too 
big  for  her  mouth,  speech  was  slurred,  and  mastication  was 
impaired.  As  a  third  example,  the  case  of  a  postman  might 
be  cited,  who  first  developed  the  weakness  in  his  legs. 

It  is  only  in  the  early  and  slight  cases  that  there  is  any 
difficulty  in  recognizing  the  condition,  and  even  in  these  cases 
the  history  and  the  facial  expression  may  suffice  to  point  to 
the  nature  of  the  disease,  and  permit  the  confirmation  of  the 
diagnosis  by  electrical  examination  and  the  discovery  of  the 
'  myasthenic  reaction.' 


DISORDERS  OF  MUSCULAR  FUNCTION 


487 


Three  types  of  the  disease  are  found  :  — 

1.  Cases  in  which  the  symptoms  are  transitory  and  the 
characteristic  facies  absent. 

2.  Cases  in  which  the  facial,  ocular,  or  bulbar  symptoms 
are  well  marked,  but  do  not  tend  to  progress,  and  in  which 
general  weakness  is  not  a  prominent  symptom. 

3.  Cases  in  which,  in  addition  to  the  facial,  ocular,  and 
bulbar  symptoms,  marked  general  weakness,  impaired  action 


Fig.  165. 


Fig.  166. 


Two  figures  illustrating  the  facial  appearance  in  a  case  of  myasthenia  gravis. 
Fig.  165  shows  external  ophthalmoplegia  on  attempting  to  look  to  the 
right.     Fig.  166  shows  the  myasthenic  smile. 


of  the  respiratory  muscles,  and  sometimes  dilatation  of  the 
heart  are  present. 

The  prognosis  as  regards  life  is  good  in  the  first  two  types, 
but  bad  in  the  third,  death  usually  occurring  suddenly  from 
respiratory  failure  within  two  years  of  the  onset  of  the 
disease.  In  all  cases  there  is  a  tendency  to  sweating  and 
vaso-motor  disturbances,  and  in  women  the  symptoms  are 
always  aggravated  by  the  catamenia. 

The  mental  condition  is  unimpaired,  except  that  myas- 
thenics are  often  highly  emotional  and  easily  upset  by  trifling 
circumstances. 

Motor  system.  Cranial  nerves.  Ptosis,  usually  bilateral 
and  more  marked  on  one  side,  is  a  common  symptom.  It  is 
more  pronounced  towards  the  evening.     The  drooping  of  the 


488  NERVOUS  DISEASES 

lids  is  to  some  extent  counteracted  by  a  tilting  of  tbe  head 
backwards,  but  the  over-action  of  the  frontalis,  so  common  in 
tabetics,  is  not  observed  in  myasthenic  patients. 

Diplopia  and  strabismus  from  weakness  of  the  external 
rectus  muscle  are  not  uncommon,  and  in  some  cases  com- 
plete ophthalmoplegia  externa  has  been  noted.  There  may, 
or  may  not,  be  nystagmoid  movements  on  extreme  deviation 
of  the  globes,  and  the  ocular  movement  is  often  ill- sustained 
and  easily  fatigued.  The  pupils  retain  their  reaction  to 
light,  and  the  accommodative  action  of  the  ciliary  muscle  is 
unimpaired.     (Fig.  165.) 

Weakness  of  the  muscles  of  mastication  is  very  common, 
and  the  mouth  is  sometimes  kept  slightly  open  from  a  falling- 
down  of  the  lower  jaw. 

On  looking  at  one  of  these  patients,  the  facial  expression 
at  once  attracts  attention.  At  first  sight  it  suggests  the 
myopathic  face.  The  forehead  is  smooth,  slight  ptosis  is 
present,  and  the  head  gently  tilted  backwards.  The  mouth 
and  lower  part  of  the  face,  when  at  rest,  give  the  impression 
of  a  face  characteristic  of  disgust,  the  upper  lip  being  slightly 
retracted,  the  corner  of  the  mouth  drooped,  and  the  lips 
parted.  On  smiling,  this  appearance  is  exaggerated,  the 
upper  lip  is  curled,  the  corners  of  the  mouth  remain  drooped, 
and  the  smile  has  the  appearance  of  a  sneer.     (Fig.  166.) 

On  testing  voluntary  power,  the  forehead  cannot  be 
wrinkled,  nor  the  eyelids  firmly  closed.  Neither  whistling 
nor  blowing  out  the  cheeks  can  be  accomplished. 

Bulbar  symptoms  consist  of  difficulty  in  swallowing,  regur- 
gitation of  fluids  through  the  nose,  and  a  nasal  tone  of  speech. 

Paralysis  of  the  vocal  cords  is  rare,  but  some  paresis  or 
weakness  of  the  abductors,  giving  rise  to  stertor,  has  been 
observed. 

The  tongue  may  or  may  not  be  affected,  being  less 
commonly  implicated  than  the  other  neighbouring  muscles. 
Its  appearance,  however,  is  characteristic.  In  rare  cases  it 
has  been  found  atrophied.  As  a  rule  it  is  soft,  flabby, 
tremulous,  indented  by  the  teeth,  and  has  three  well-marked 
furrows  in  the  longitudinal  direction,  one  median  and  two 
lateral. 

Speech  may  be  normal  or  slightly  nasal  in  tone.  Where 
any    defect  exists,  it    may   be    accentuated    and   made   more 


DISORDERS  OF  MUSCULAR  FUNCTION  489 

obvious  by  inducing  exhaustion  from  repetition  of  words  or 
phrases. 

Trunk  muscles.  The  muscles  of  the  neck  are  often  im- 
plicated at  an  early  stage.  Owing  to  this,  the  head  tends  to 
fall  forwards  or  backwards.  Both  the  back  and  abdominal 
muscles  are  affected,  and  more  especially  the  intercostals 
and  other  respiratory  muscles,  so  that  dyspnoea  on  exertion 
and  difficulty  in  breathing  are  frequent. 

The  extremities.  The  most  marked  weakness  is  found 
in  the  shoulder  muscles.  This  is  readily  brought  out  by 
making  the  patient  maintain  the  arms  abducted  horizontally 
from  the  side,  if  necessary  against  resistance.  This  leads  to 
rapid  fatigue,  and  finally  inability  to  raise  the  arms  at  all. 
The  grasps  are  feeble  and  may  be  easily  exhausted.  As  in  the 
arms,  so  also  in  the  lower  limbs,  the  proximal  muscles,  espe- 
cially the  flexors  of  the  hip  joint,  are  readily  affected.  This, 
in  conjunction  with  general  weakness  of  the  lower  limbs,  gives 
rise  to  a  peculiar  combination  of  waddling  and  steppage  gait. 
In  all  severe  cases,  where  such  deficiencies  exist,  dyspnoea  on 
exertion  effectually  hinders  progression.  This  weakness  is 
most  marked  in  the  muscles  chiefly  employed,  local  effort 
producing  not  only  local  exhaustion,  but  also  an  abnormal 
degree  of  general  muscular  fatigue.  Muscular  weakness  gives 
rise  to  subjective  sensations  of  aching,  pain,  and  dragging. 

The  myotatic  irritability  is  increased  in  duration,  but  re- 
duced in  briskness.  Thus  a  tap  on  a  muscle  may  induce  a 
slow  but  well-sustained  muscular  reaction. 

The  electrical  reactions.  A  tetanising  faradic  current 
induces  at  first  a  brisk  muscular  response,  which,  however, 
tends  to  fade  and  finally  disappears  on  the  continuance  of  the 
current.  On  reapplying  the  electrodes  after  a  period  of  rest, 
a  good  contraction  is  again  obtained,  which  also  eventually 
disappears.  This  reaction  is  not  obtained  by  the  galvanic 
current. 

Similar  results  may  be  obtained  by  isolated  faradic  shocks, 
but  the  application  of  rapid,  interrupted  galvanic  shocks  failed 
to  produce  any  exhaustion,  at  least  in  two  cases  in  which  it 
was  tried.  Exhaustion  of  the  muscles  by  faradic  currents,  in 
the  majority  of  cases,  induces  a  weakness  of  voluntary  effort  ; 
and  fatigue  induced  by  voluntary  effort  certainly  causes  a 
more  rapid  faradic  exhaustion. 


490  NERVOUS  DISEASES 

The  above-described  myasthenic  reactions  vary  to  a  great 
extent,  both  from  day  to  dajr  and  in  different  muscular  groups. 
It  is  most  characteristic  and  constant  in  the  facial  muscles. 

The  absence  of  a  myasthenic  reaction  does  not  negative  the 
existence  of  this  disease. 

Sensory  system.  There  is  no  loss  of  sensation,  but  sub- 
jective feelings  of  aching  and  pain  in  association  with  muscular 
exhaustion  are  not  uncommon.  Occasionally  slight  muscular 
hyperesthesia  to  deep  pressure  has  been  met  with  (Buzzard). 

Reflexes.  The  deep  reflexes  are  brisk,  normal  or  sub- 
normal, and  in  severe  cases  may  be  exhausted  by  repeated 
tapping.  The  superficial  reflexes  are  usually  increased.  The 
plantars  are  of  the  flexor  type. 

The  sphincters  are  never  affected. 

Vaso-motor  functions.    Flushing  and  sweating  are  common. 

In  severe  cases  dilatation  of  the  heart  occurs  with  the 
development  of  an  apical  systolic  murmur,  but  this  condition 
may  vary  from  time  to  time. 

Respiratory  complications  are  invariably  fatal. 

Pregnancy  may  occur  and  labour  be  accomplished  normally, 
but  their  influence  upon  the  disease  is  unfavourable. 

Diagnosis.  Myasthenia  has  to  be  distinguished  from 
several  conditions,  of  which  the  more  important  are  hysteria, 
the  myopathies,  bulbar  paralysis  and  tumour  of  the  pons. 

1.  Hysteria.  The  slighter  forms  of  myasthenia  may 
simulate  lrysteria,  but  the  distribution  of  the  weakness,  its 
onset  after  fatigue  and  towards  evening,  the  absence  of 
sensory  loss  or  of  hysterical  seizures,  and  the  presence  of  the 
myasthenic  reaction  are  usually  sufficient  guides. 

2.  Muscular  dystrophy.  Myasthenia  is  only  liable  to  be 
confused  with  the  facio-scapular  type,  and  is  to  be  dis- 
tinguished from  it  by  the  absence  of  muscular  atrophy  and 
hypertrophy,  by  the  relapsing  character  of  the  weakness,  and 
by  the  presence  of  the  myasthenic  reaction.  The  character- 
istic degenerative  electrical  changes  of  the  muscular  dystrophies 
are  absent. 

3.  From  bulbar  palsy  and  from  focal  lesions  of  the  pons 
and  medulla  it  is  distinguished  by  the  absence  of  atrophy, 
the  age  of  the  patient,  and  the  remission  of  the  symptoms 
after  fatigue. 

4.  From  chronic  external  ophthalmoplegia,  by  the  history  of 


DISORDERS  OF  MUSCULAR  FUNCTION  491 

the  onset,  by  the  affection  of  other  parts  of  the  body,  by 
the  influence  of  fatigue,  by  the  remittent  character  of  the 
symptoms,  and  by  the  presence  of  the  myasthenic  reaction. 

Prognosis.  The  outlook  is  bad  as  regards  recovery  from 
the  disease. 

In  the  milder  forms  the  duration  of  life  is  quite  uncertain, 
but  patients  have  been  known  to  live  for  fifteen  years  after  the 
onset  of  the  symptoms. 

In  acute  cases,  especially  those  in  which  attacks  of  re- 
spiratory difficulty  are  common,  the  duration  of  life  rarely 
exceeds  two  years. 

Cases  presenting  the  symptoms  of  the  disease  in  a  well- 
marked  form  may,  with  care  and  the  avoidance  of  exertion, 
live  for  an  indefinite  number  of  years. 

Treatment.  Rest  should  be  advocated,  and  in  the  severe 
cases  rest  in  bed  is  essential.  In  addition,  general  tonics> 
especially  strychnine  hypodermically,  are  recommended. 
Warm  clothing  and  the  avoidance  of  chills  and  respiratory 
complications  are  important.  All  forms  of  electrical  applica- 
tions and  massage  should  be  avoided.  Sodium  sulphite 
deserves  a  wider  trial. 


PARAMYOCLONUS  MULTIPLEX 

This  is  a  rare  condition,  characterised  by  the  repeated 
occurrence  of  short,  sharp,  shock-like  clonic  contractions  of 
single  muscles  or  portions  of  muscles,  more  rarely  of  groups  of 
muscles.  The  contractions  resemble  those  produced  by  the 
application  of  a  faradic  current.  They  may  occur  in  any  of 
the  muscles  of  the  body,  but  predominate  in  the  supinator 
longus,  biceps,  trapezius,  pectorals,  recti  abdominales,  quadri- 
ceps extensor  cruris,  and  semi-tendinosus. 

The  mental  state  is  not  affected.  The  contractions  do 
not  give  rise  to  pain,  but  occasion  great  distress  from  their 
inconvenience. 

The  condition  is  bilateral,  and  a  muscle  on  one  side  is  often 
observed  to  contract  immediately  after  its  fellow7  of  the  opposite 
side.  The  shock-like  movements  are  increased  on  emotion  and 
disappear  during  sleep.  They  do  not  interfere  with  active 
movements.  They  may  occur  at  different  times  in  different 
parts  of  the  body.     The  rate  of  contraction  varies  widely,  but 


492  NERVOUS  DISEASES 

as  many  as  a  hundred  contractions  may  be  observed  in 
a  minute. 

On  examination,  apart  from  the  clonic  tremor,  the  motor 
functions  are  normal.  The  sensory  system  is  unaffected. 
Handling  the  limbs  may  increase  the  contractions.  The  deep 
and  superficial  reflexes  are  increased.  There  is  no  sphincter 
trouble.    The  electrical  irritability  of  the  muscles  is  unaltered. 

The  condition  has  arisen  after  trauma,  acute  infective 
diseases,  and  fright. 

The  prognosis  is  grave  as  regards  recovery. 

An  hysterical  type  has  been  described,  in  which  the 
movements  are  of  larger  range,  affecting  groups  rather  than 
single  muscles,  and  not  involving  bundles  of  fibres.  This 
condition  is  probably  quite  distinct  from  the  true  form,  and 
the  prognosis  is  good.  There  may  be  other  associated 
hysterical  stigmata. 

A  combination  of  myoclonus  and  epilepsy  occurring  in 
families  has  been  described  by  Unverricht.  In  it  the  spasm 
affects  the  muscles  of  the  body,  limbs,  tongue,  pharynx, 
palate,  and  diaphragm.  This  type  has  also  been  observed 
unassociated  with  epilepsy,  but  with  mental  enfeeblement  and 
backwardness. 


Part   XIII 

DISEASES  OF  OBSCURE  ORIGIN  CHARAC- 
TERISED CHIEFLY  BY  DISORDERS  OF 
MOTION 

Chapter  I 
PARALYSIS     AGITANS 

[syn.  :  Parkinson's  disease] 

This  is  a  progressive  disease,  usually  characterised  by 
muscular  rigidity,  which  gives  rise  to  a  mask-like  expression 
of  face  and  a  typical  attitude  and  gait,  but  frequently 
attended  by  the  presence  of  a  rhythmic  tremor. 

Etiology.  The  disease  commences  most  commonly 
between  the  ages  of  forty  and  seventy,  the  majority  of  cases 
arising  in  the  middle  period  between  fifty  and  sixty.  It  is  rare 
under  forty  years,  but  cases  are  from  time  to  time  seen  in 
which  the  symptoms  have  arisen  under  thirty. 

Heredity  plays  little  or  no  part  in  its  causation.  The 
hereditary  feature,  which  we  have  found  most  constant,  is  a 
tendency  towards  longevity  in  one  or  both  parents.  In  a 
number  of  cases  in  which  this  factor  was  ascertained,  the  age 
at  death  of  one  or  other  parent  ranged  from  seventy  to  ninety 
years. 

There  is  no  doubt  that  severe  mental  strain,  shock,  and 
worry  have  an  important  influence  in  its  causation,  many  cases 
commencing  definitely  after  the  strain  of  nursing  relatives 
and  the  anxiety  of  the  death-bed  scene. 

Traumatism  may  also  play  a  part,  and  sometimes  deter- 
mines the  limb  in  which  the  tremor  starts. 

There  is  an  occasional  relation  between  vascular  degene- 
ration and  the  onset  of  the  disease. 

493 


494  NERVOUS  DISEASES 

Pathology.  No  special  or  constant  pathology  of  paralysis 
agitans  is  known,  although  various  morbid  changes  have  been 
found  in  those  dying  of  this  disorder,  such  as  degenerative 
changes  in  the  walls  of  the  smaller  arterioles,  pigmentation 
and  vacuolation  of  the  ganglion  cells,  and  increase  of  the 
neuroglial  tissue.  But  these  alterations  are  found  in  other 
conditions,  and  are  usually  the  result  of  senile  changes. 

The  absence  of  definitely  degenerative  alterations  in  the 
muscles,  and  the  onset  of  the  tremor  occasionally  as  a  result 
of  nervous  shock,  and  sometimes  of  injury,  would  seem  to 
suggest  a  psychical  basis  of  the  symptoms  in  some  cases.  On 
the  other  hand,  evidence  has  been  advanced  by  some 
writers  to  show  that  the  disease  is  primarily  a  muscular 
condition. 

Symptoms.  Although  the  term  '  paralysis  agitans  '  sug- 
gests tremor  as  the  outstanding  feature  of  the  disease,  this 
symptom  is  in  reality  only  one  of  its  several  manifestations, 
and  is  by  no  means  an  early  sign.  Two  chief  types  are 
found  : — 

(a)  A  type  characterised  by  the  early  appearance  of 
rigidity,  which  shows  itself  primarily  in  the  expressionless 
face  and  the  well-known  attitude  and  gait.  This  is  usually 
accompanied  by  paresthesia,  subjective  sensations  of  heat  and 
cold,  and  aching  and  stiffness  of  the  muscles,  chiefly  of  the 
neck  and  shoulders, 

(b)  The  other  and  less  common  type  commences  with 
either  slight  or  well-marked  tremor,  unattended  by  rigidity 
or  sensory  symptoms. 

In  most  cases  the  first  symptom  is  rigidity,  to  which 
tremor  is  added  in  the  later  stages. 

The  mental  condition  is  not  usually  impaired,  but  attacks 
of  despondency  and  depression  are  not  uncommon.  This 
mental  attitude  is  frequently  accentuated  by  the  lack  of 
expression  on  the  patient's  face,  which  is  characterised  by  a 
mask-like  appearance  and  a  failure  of  emotional  display. 

Cranial  nerves.  The  most  striking  feature  on  examining 
the  cranial  nerves  is  the  fixed,  intense,  and  anxious  facial 
expression,  arising  from  the  rigidity  of  the  facial  muscles 
and  the  wiping  out  of  the  emotional  movements.  In  some 
cases  tremor  of  the  lower  jaw  and  tongue  are  observed.  The 
articulation  tends  towards  a  monotone,  and  although  each 


DISORDERS  OF  MOTION 


105 


word  is  clearly  and  distinctly  pronounced,  there  is  no  in- 
flexion. In  the  later  stages  the  voice  is  high  pitched,  giving 
rise  to  '  the  piping  voice.'  The  ocular  movements  are  good, 
but  in  some  cases  weakness  of  the  convergent  action  of  the 
eyeballs  has  been  noted. 

Motor  system.  Eigidity,  not  amounting  to  spasticity,  is 
the  chief  feature.  The  attitude  is  quite  characteristic  of  the 
malady.   Weakness  and  difficulty  of  movement  are  associated 


[ 

"■ '-;*&■ 

H 

!if .   #^%% 

f&toh^ 

« 

■Jkf:' 

•I 

gnH&              a 

JK' 

WmmSSSBESm^Bmi 

Fig.  167. — The  face  in  a  case  of  paralysis  agitans. 


with  rigidity  and  directly  due  to  it.  The  muscular  actions  are 
slow  and  the  grasps  feeble.  Motor  weakness  is  slight  and 
rarely  advances  to  paralysis,  and  even  in  the  late  stages  some 
movement  is  retained.  The  muscles  retain  their  normal  volume 
and  their  electrical  irritability. 

Eigidity.  This  is  the  characteristic  feature  of  the  disease. 
It  is  usually  the  earliest  symptom,  and  affects  the  thumb  or 
forefinger  before  any  tremor  makes  its  appearance.  Cases 
of  this  early  type  usually  present  considerable  difficulty  in 
the  diagnosis.     (Fig.  168.) 

To  the  rigidity  are  ascribed  many  of  the  most  typical 
features  of  the  malady,  such  as  the  mask-like  facial  appear- 
ance, the  forward  position  of  the  head,  the  rigid  and  curved 


l!Mi 


NERVOUS   DISEASES 


back,  and  the  posture  of  the  hands  with  the  fingers  flexed 
at  the  metacarpophalangeal  and  extended  at  the  inter- 
phalangeal  joints.  The  appearance  of  the  hands  closely 
resembles  that  seen  in  rheumatoid  arthritis,  a  condition 
which  is  not  rarely  associated  with  it.  The  legs  are  slightly 
flexed  at  the  knees  and  the  thighs  adducted,  and  a  tendency 
to  talipes  may  develop. 

In    consequence  of    this    attitude,    the   gait   is    slow   and 

hesitating  ;  having 
started,  however,  the 
patient  may  progress  with 
increasing  rapidity,  as 
if  he  were  running  after 
his  centre  of  gravity 
(festination) .  In  some 
instances  the  converse 
condition,  in  which  there 
is  a  tendency  to  run 
backwards,  has  been  ob- 
served (retropulsion). 

Tremor.  This  is  coarse 
and  rhythmical,  charac- 
terised by  alternating 
opposition  and  extension 
of  the  thumb,  or  flexion 
and  extension  of  the  fore 
or  other  fingers,  produc- 
ing the  well-known  'cigar- 
ette rolling '  or  '  bread 
crumbling '  movements. 
Other  movements  are 
flexion  and  extension  at  the  metacarpo-phalangeal  joints, 
or  at  the  wrist,  or  pronation  and  supination  movements  of  the 
forearm,  or  all  these  movements  may  be  combined  at  one 
time.  Such  movements  may  be  seen,  from  a  slight  but 
characteristic  tremor  up  to  marked  shaking  of  the  whole  arm. 
In  the  lower  limb  the  tremor  may  be  flexion  and  exten- 
sion at  the  ankle  joint,  so  as  to  produce  a  tapping  sound 
when  the  foot  is  at  rest  upon  the  floor.  Movements  of  the 
lower  jaw  are  less  frequently  observed,  and  are  usually  due 
to  definite  tremors  in  the  nrylohyoid  and  digastric  muscles. 


Fig.  168. — Characteristic  attitude  of 
paralysis  agitans. 


DISORDERS  OF  MOTION 


407 


To-and-fro  movements  of  the  head  are  sometimes  seen,  but 
rotation  movements  are  rare.  Tremor  may  also  implicate 
the  lips  and  tongue. 

The  special  characters  of  this  tremor  are,  in  addition  to 
its  rhythmic  and  usually  coarse  features,  its  continuance 
during    repose,    and   its   temporary    suspension   during   the 


Fig.  169. 


Fig.  170. 


Figs.  169  and  170. — Two  figures  illustrative  of  the  characteristic  attitude  of 
the  head,  limbs,  arid  body  in  paralysis  agitaus. 


execution  of  voluntary  movements.  It  may  also  be  tempo- 
rarily arrested  by  an  effort  of  will.  Arrest  of  the  movements 
in  one  limb  may  induce  an  exaggerated  tremor  in  another 
limb.  In  the  later  stages,  however,  the  tremor  cannot 
be  voluntarily  arrested,  and  is  sometimes  found  to  show 
exaggeration  on  volitional  and  emotional  movements. 

The   onset  of  the  tremor  is  usually  insidious,  and  most 

32 


498  NERVOUS  DISEASES 

commonly  in  the  thumb  or  fingers.  If  it  commences  in  the 
upper  limb,  the  lower  limb  upon  the  same  side  is  involved 
before  the  limbs  upon  the  opposite  side.  It  affects  the 
proximal  rather  than  the  distal  portions  of  the  lower  limb. 

In  those  cases  in  which  tremor  is  an  early  and  prominent 
feature,  rigidity  is  rare,  and  the  characteristic  facial  expres- 
sion and  attitude  are  late  in  appearing,  or  even  absent.  In 
this  type  the  range  or  excursion  of  the  tremor  is  larger  and 
more  irregular. 

Complaint  is  made  of  various  paresthesia.    Amongst  these 


^^i 

P^3i 

r ,  I^^H 

'  's4&t               ■ 

\    yM 

^36 

;  j  m 

3a^ 

K 

Fig.  171. — The  appearance  and  position  of  the  hands  in 
paralysis  agitans. 

may  be  mentioned  abnormal  sensations  of  heat  and  cold 
unassociated  with  any  rise  of  temperature,  and  aching  sensa- 
tions about  the  limbs  and  back  of  the  neck. 

The  deep  reflexes  are  brisk,  but  ankle  clonus  is  never 
present.  The  superficial  reflexes  are  unchanged  and  the 
plantar  is  of  the  flexor  type. 

Sphincter  control  is  retained  to  the  end. 

Restlessness  at  night,  sometimes  accompanied  by  insomnia, 
may  be  present. 

The  differential  diagnosis  has  to  be  made  from  senile 
tremor,  double  hemiplegia,  bilateral  cortical  degeneration,  and 
focal  lesions  of  the  mid-brain. 

Senile  tremor  is  characterised  by  its  occurrence  in  extreme 
old    age,    its   irregularity,    its    bilateral    distribution,    by    an 


DISORDERS  OF  MOTION  499 

absence  of  rigidity,  and  by  the  early  involvement  of  the 
head. 

Double  hemiplegia  is  distinguished  by  the  presence  of 
reflex  changes  characteristic  of  organic  disease,  notably  the 
extensor  plantar  response,  by  the  excess  of  weakness  over 
rigidity,  and  by  the  absence  of  tremor  and  the  presence  of 
exaggerated  emotional  display. 

Bilateral  cortical  degeneration  most  closely  resembles 
paralysis  agitans,  inasmuch  as  a  slowly  progressive  rigidity 
with  motor  weakness  is  present.  Although  the  facial  expres- 
sion has  a  superficial  resemblance  to  paralysis  agitans,  on 
closer  examination  the  mask-like  expression  is  seen  to  be  due 
to  fixation  of  the  normal  lines.  The  reflexes  are  at  first 
normal,  as  in  paralysis  agitans,  but  eventually  become  charac- 
teristic of  organic  disease.  The  mental  condition  shows  a 
steady  deterioration,  with  spasmodic  laughing  and  crying. 

In  Focal  lesions  of  the  mid-brain  the  onset  is  sudden,  the 
symptoms  are  unilateral,  impairment  of  the  upward  movement 
of  the  eyes  and  of  the  pupillary  light  reaction  are  present,  and 
a  tendency  towards  subsidence  of  the  symptoms  develops  in 
course  of  time. 

Prognosis.  It  is  a  chronic  progressive  disorder,  of  long 
duration,  and  having  little  tendency  to  cause  death.  It  has 
been  known  to  last  from  fifteen  to  thirty  years,  but  the  average 
duration  is  from  ten  to  twelve  years.  Death  is  usually  due  to 
an  intercurrent  malady,  such  as  malignant  disease,  pulmonary 
complications,  or  exhaustion. 

Treatment.  Paralysis  agitans  is  a  disease  for  which 
little  can  be  done,  even  of  a  temporary  character.  In  cases 
characterised  by  rigidity,  massage,  passive  movements,  and 
warm  baths  are  of  some  use.  In  the  tremulous  forms,  mild 
faradism  applied  to  the  limbs  which  are  the  seat  of  tremor  is 
often  beneficial. 

Of  drug  treatment  we  have  found  most  benefit  from  daily 
hypodermic  injections  of  hydrobromate  of  hyoscine  (gr.  ^ 
to  tij),  especially  in  the  early  stages  and  in  patients  under 
fifty  years  of  age.  Few  other  drugs  are  of  much  service. 
Arsenic  and  strychnine  are  probably  of  most  use  in  improving 
the  general  condition  and  relieving  pain  and  despondency. 
Temporary  benefit  may  be  derived  from  the  bromides,  chloral, 
and  cannabis  indica. 

32   * 


500  NERVOUS  DISEASES 

In  those  cases  in  which  the  gait  tends  to  become  festinant, 
the  patient  should  be  trained  to  take  long  steps  by  means 
of  carefully  regulated  walking  exercises.  This  will  often 
materially  improve  the  gait  and  enable  the  patient  to  get 
about  with  considerable  freedom  and  comfort. 

In  view  of  the  age  at  which  the  disease  commences,  it 
would  seem  advisable  to  try  the  effect  of  the  organic  extracts 
— thyroid,  pituitary,  ovarian,  and  testicular. 


Chapter  II 
CHOREA 

(SYN.  :     ST.    VITUS*  S    DANCE.       SYDENHAM'S    CHOREA) 

Chorea  is  a  disease  chiefly  affecting  children  and  young 
adults,  intimately  associated  with  rheumatic  manifestations, 
and  characterised  by  psychical  changes  and  motor  disturbances, 
the  latter  consisting  of  irregular,  jerking,  purposeless,  semi- 
purposive,  involuntary  movements. 

Etiology.  Females  are  more  frequently  affected  than 
males,  in  the  proportion  of  about  three  to  one.  Its  occur- 
rence is  most  common  between  the  ages  of  five  and  fifteen. 
It  is  rare  after  twenty,  except  when  associated  with  pregnancy. 
A  form  occurring  in  old  age,  and  known  as  senile  chorea, 
has  been  described.  It  is  much  more  common  in  hospital 
than  in  private  practice.  Its  seasonal  incidence  is  in 
striking  correspondence  with  that  of  rheumatism,  the  period 
of  greatest  frequency  commencing  in  December,  reaching  a 
maximum  in  March,  falling  in  April,  and  showing  a  further 
rise  in  May,  from  which  month  a  steady  decline  takes  place 
to  November. 

A  family  disposition  towards  rheumatism  and  nervous 
affections  are  striking  features  in  many  cases.  The  patients 
are  of  the  sanguine  temperament. 

Although  emotion  and  fright  have  been  extensively  quoted 
as  exciting  causes,  careful  inquiry  will  bring  to  light  the  fact 


DISORDERS  OF  MOTION  501 

that  some  signs  of  the  disease  had  been  previously  observed. 
Amongst  other  ascribed  causes,  imitation,  trauma,  reflex  irrita- 
tion, and  eye-strain  are  mentioned,  but  while  these  may  have 
some  influence  in  aggravating  the  symptoms,  they  are  purely 
accidental  and  in  no  sense  causal  influences. 

The  relation  between  rheumatism  and  chorea  was  pointed 
out  in  France  and  in  our  own  country  many  years  ago ;  but 
attention  has  been  lately  redirected  to  it,  and  during  the  past 
few  years  observations  have  been  made  establishing  an 
etiological  relation  between  the  two  disorders.  Chorea  may 
precede,  coincide  with,  or  follow  acute  rheumatism.  In  many 
cases  the  association  is  indisputable,  but  in  others  vague 
pains  and  sore  throat  are  the  only  previous  rheumatic 
symptoms. 

An  analysis  of  our  cases  shows  that  out  of  58  cases  of 
chorea  minor  only  6,  or  10*3  per  cent.,  were  without  any 
personal  or  hereditary  history  or  sign  of  rheumatism.  Of  the 
remainder  17,  or  29  per  cent.,  gave  a  history  of  rheumatic 
fever  preceding  the  first  attack  of  chorea,  15,  or  25*8  per 
cent.,  gave  an  account  of  rheumatic  pains,  while  12,  or  20-7 
per  cent.,  although  presenting  no  personal  sign  or  history  of 
rheumatism,  gave  a  history  of  rheumatism  or  chorea  in  their 
family.  In  two  cases,  in  which  there  was  no  personal  or 
family  history  of  chorea  or  rheumatism,  valvular  disease  of 
th  e  heart  was  detected  on  auscultation. 

A  diplococcus,  identical  with  that  found  by  Poynton  and 
Paine  l  in  the  blood  and  valvular  vegetations  in  acute  rheu- 
matism, has  been  observed  in  the  cerebral  cortex,  blood,  and 
valves  of  the  heart  in  cases  of  fatal  chorea.  The  organism 
has  also  been  obtained  during  life  from  the  cerebro-spinal 
fluid  in  cases  of  chorea.  This  association  is  striking,  and  is 
made  more  so  by  the  finding  of  the  same  organism  in  the 
brains  of  fatal  cases  of  chorea  occurring  during  pregnancy 
(Paine,  Poynton,  and  Holmes). 

Infectious  disorders  do  not  appear  to  play  any  active  part 
in  the  production  of  chorea,  except  perhaps  scarlet  fever.  On 
the  other  hand,  the  occurrence  of  an  acute  disorder  during  the 
course  of  chorea  may  induce  a  lessening  of  the  disease. 

Chorea  and  epilepsy.     An  intimate  relation  seems  to  exist 

1  Poynton  and  Paine,  Lancet,  1901 ;  Brain,  1905. 


502  XKIlYOrs   DISEASES 

between  chorea  and  epilepsy,  but  it  is  more  common  to  find 
epilepsy  mentioned  in  the  family  history  of  choreics  than  the 
reverse.  Both  chorea  and  epilepsy  may  predispose  towards 
each  other  ;  and  in  neuropathic  families  epilepsy  and  chorea 
may  be  present  in  different  members.  Epilepsy  and  chorea 
may  also  be  present,  either  simultaneously  or  at  different 
times,  in  the  same  person.  A  history  of  infantile  convulsions 
is  not  uncommon  in  those  suffering  from  chorea. 

Symptomatology.  The  symptoms  of  the  disease  usually 
set  in  more  or  less  gradually,  and  are  at  first  of  a  psychical 
nature.  A  change  is  noticed  in  the  child's  behaviour.  He 
shows  irritability  of  temper  and  peevishness,  while  his  manner 
is  nervous  or  apathetic.  At  this  stage  the  malady  is  not 
recognised,  and  consequently  the  child  is  scolded  or  punished. 
He  then  becomes  emotional  and  has  fits  of  crying  or  of  temper, 
with  occasionally  night  -  terrors  and  restlessness.  He  is 
ndget}r  and  clumsy,  especially  at  table  or  when  under  observa- 
tion. Complaint  may  be  made  of  headache  or  of  pains  in 
the  limbs,  especially  the  legs,  followed  by  the  typical  move- 
ments of  chorea,  which  explain  the  previous  behaviour  and 
symptoms.  The  hands  are  usually  earliest  affected,  then  the 
face,  and  later  the  legs.  Very  often  one  side  is  affected  earlier 
and  more  extensively  than  the  other,  but  as  a  rule  the 
movements  are  bilateral,  although  in  some  cases  they  remain 
one-sided  (hemichorea\ 

The  facial  appearance  is  characteristic.  In  nervous  and 
high-spirited  children  the  expression  is  one  of  overflowing 
nervousness,  the  eyes  are  bright  and  glistening,  the  face  is 
continually  being  twisted  into  grimaces  and  is  never  still. 
The  head  is  jerked  about,  and  articulation  may  be  impaired, 
especially  when  the  right  side  is  involved.  If  the  child  is 
standing,  the  arms  are  kept  out  of  sight  behind  the  back.  The 
shoulders  are  wriggled,  and  the  hands  are  seen  to  be  jerked 
about  with  irregular,  rapid,  and  in  co-ordinated  movements. 
These  movements  are  not  clonic  or  confined  to  one  muscle, 
but  are  the  result  of  contractions  of  groups  of  muscles, 
and  have  a  purposive  character,  though  their  aim  is  pur- 
poseless. Mental  excitement  or  attempts  to  perform  fine 
movements  result  in  their  exaggeration. 

In  severer  cases  the  movements  are  continuous,  the  patient 
being  unable  to  walk  or  to  lie  still,  and  presenting  a  constant 


I)I,S0M)KRS  OF  MOTION  503 

whirlpool  of  restless  motion.     Ho  has  a  wild,  restless  look,  and 
herpes  may  be  seen  on  the  lips. 

The  maniacal  cases  are  rare  in  children,  but  are  more 
common  in  young  adults  and  in  pregnancy.  As  a  rule  they 
develop  gradually  out  of  a  simple  case,  the  patient  either  first 
becoming  dull  or  heavy,  with  delusions,  mental  excitement, 
and  inarticulate  incoherent  speech,  or  the  primary  restless 
state  passes  directly  into  one  of  delirium  and  mania.  The 
patient  is  sleepless,  and  tosses  about  wildly  in  bed,  with  con- 
tinuous movements  of  all  parts  of  the  body.  In  these  cases 
the  temperature  may  rise  to  hyperpyrexia. 

The  mental  condition  frequently  shows  some  sign  of  impair- 
ment during  the  course  of  the  malady.  The  facial  expression 
is  vacant,  there  is  a  failure  of  the  power  of  attention,  and 
often  a  want  of  emotional  stability.  In  the  early  stages, 
irritability,  obstinacy,  and  waywardness  may  reveal  the  fact 
that  the  child  is  not  in  its  usual  health.  A  form  of  chorea — 
chorea  insaniens — is  characterised  by  psychical  features  of  an 
acute  type,  incoherence  of  speech,  delusional  and  hallucinatory 
symptoms,  and  the  usual  phenomena  of  an  acutely  maniacal 
state. 

Motor  system.  The  most  obvious  affection  is  the  occur- 
rence of  the  characteristic  involuntary  movements  already 
described.  It  may  be  stated  that  in  the  majority  of  cases  the 
movements,  which  are  rapid  and  jerky  in  character,  are  not 
truly  clonic,  as  described  by  some  writers.  They  are  the 
consequence  of  the  inco-ordinated  action  of  groups  of  muscles, 
and  as  such  are  purposive  in  appearance,  though  purposeless 
in  effect.  It  is  true  that  in  the  face  a  twitching  movement  of 
one  or  more  muscles  may  be  observed,  not  amounting  to  a 
grimace,  and  it  is  not  uncommon  for  tic-like  movements  to 
develop  out  of  an  attack  of  chorea  and  to  persist  for  some 
time.  These  are  not  genuine  choreic  movements,  but  exhibit 
all  the  features  of  a  tic  (p.  584). 

Choreic  movements  are  not  limited  to  any  particular 
muscular  group,  but  are  observed  now  in  one  place,  now  in 
another.  Voluntary  action  may  be  well  carried  out,  provided 
that  the  performance  of  the  act  is  not  interfered  with  by  the 
occurrence  of  involuntary  movement.  Eealising  this,  the 
patient  carries  out  the  action  hurriedly,  in  order  to  effect  his 
purpose  during  a  quiescent  period. 


504 


NERVOUS  DISEASES 


The  movements  of  chorea  are  increased  by  emotion  and 
excitement,  and  in  the  slighter  cases  subside  during  sleep. 

Motor  power  is  impaired,  and  fatigue  is  easily  induced. 
In  some  cases,  especially  in  the  unilateral  types,  this  weakness 
is  well  marked,  and  is  associated  with  the  symptoms  of  an 
organic  affection  of  the  motor  path.  In  one  type  of  the 
malady — chorea  mollis — an  almost  complete  loss  of  power 
exists  ;  in  these  cases  voluntary  movement  is  slight  or  absent, 
hypotonia  is  present,  and   the  deep  reflexes    are  abolished. 

This  type  of  the  malady 
is  usually  found  in 
children  of  lymphatic 
temperament. 

The  muscles  are  well 
developed,  there  is  no 
atrophy,  but  a  tend- 
enc^y  to  hypotonia  is 
constant.  The  hand 
in  chorea  frequently 
shows  a  tendency  to- 
wards hyper-extension 
of  the  proximal  phal- 
anges. (Fig.  172.) 
The  reflexes.  The 
deep  reflexes  may  be  normal,  exaggerated,  or  absent.  A 
slight  increase  is  the  most  common  deviation  from  the 
normal.  A  more  or  less  characteristic  phenomenon  is  an 
almost  tonic  prolongation  of  the  jerk,  the  response  to  the 
tap  being  slightly  delayed,  and  the  effect  prolonged.  They 
may  be  abolished  in  the  paralytic  form. 

The  superficial  reflexes  are  brisk,  but  in  the  hemiplegic 
cases  they  may  be  lost  or  diminished  on  the  affected  side.  In 
these  cases  an  extensor  plantar  response  is  sometimes  found. 
Sensation.  There  are  no  objective  sensory  changes,  but 
pains  in  the  limbs  and  joints  frequently  precede  or  accom- 
pany the  choreic  movements. 

The  car dio -vascular  system.  An  organic  affection  of  the 
valves  of  the  heart,  more  especially  the  mitral  cusps,  is 
especially  frequent  in  chorea.  According  to  Gowers,  90  per 
cent,  of  the  fatal  cases  exhibit  valvular  disease  and  endocar- 
ditis.    We  found  50  per  cent,  of  the  ordinary  cases  of  chorea 


Fig.  172. — Illustrates  a  not  uncommon  ap- 
pearance of  the  outstretched  hands  in  a 
case  of  chorea. 


DISORDERS  OF  MOTION  505 

minor,  examined  in  hospital  out-patient  practice,  with  valvular 
murmurs. 

Although  less  common  than  endocarditis,  the  association 
of  chorea  with  pericarditis  has  long  heen  known. 

In  the  cases  of  chorea  minor  which  recover  satisfactorily 
systolic  murmurs  are  not  uncommon,  hut  in  many  cases 
these  are  of  a  ha3mic  character.  In  all  cases,  however,  the 
heart  should  be  carefully  watched  during  the  progress  of  the 
malady.  The  rarity  of  aortic  disease,  as  compared  with 
mitral  regurgitation — an  observation  to  which  attention  has 
been  directed  by  other  writers — was  fully  confirmed  in  our  own 
series  of  cases. 

The  pulse  may  be  irregular  or  intermittent,  and  is  usually 
increased  in  frequency. 

Various  cutaneous  rheumatic  affections  may  also  coexist, 
such  as  erythema  nodosum  and  purpura  rheumatica.  Some 
degree  of  pyrexia  is  found  in  the  severe  cases,  but  in  the 
common  type  of  chorea  minor  the  temperature  is  normal. 
When  the  temperature  is  raised,  endocarditis  or  other  cardiac 
complication  is  present. 

Course  and  duration.  Chorea  is  essentially  a  relapsing 
malady,  the  number  of  attacks  varying  from  two  up  to  six  or 
more  over  a  number  of  years.  In  some  cases  the  movements 
never  entirely  disappear  during  the  intervals.  The  frequency 
of  periodic  attacks  may  be  seen  from  the  following  table,  which 
shows  the  number  of  relapses  in  a  series  of  fifty-eight  cases  of 
chorea  minor : — 

34  cases  with     1  attack. 


13 

>j 

>j 

2 

attacks 

4 

33 

3  3 

3 

?; 

5 

33 

33 

4 

33 

1 

case 

33 

5 

3  > 

1 

33 

33 

8 

33 

58 

cases 

J? 

105 

3) 

The  duration  of  an  average  attack  is  commonly  about 
three  months,  during  which  period  it  runs  its  course  of 
incidence,  full  development,  and  gradual  subsidence. 

Death  may  be  due  to  exhaustion  arising  from  the  intensit}T 


506  NERVOUS  DISEASES 

of  the  motor  disturbance  and  the  difficulty  in  maintaining  the 
strength  by  suitable  nourishment,  or  from  such  complications 
as  endocarditis,  pericarditis,  embolism,  or  hyperpyrexia. 

Treatment.  Rest  in  bed  and  ample  nourishment  are  the 
two  main  factors  guiding  the  management  of  all  cases  of 
chorea,  whether  slight  or  severe.  Drugs  may  also  be  pre- 
scribed with  great  advantage. 

Rest  in  bed,  or,  if  the  movements  are  severe,,  upon  a 
mattress  on  the  floor  in  the  corner  of  the  room,  should  be 
maintained  until  the  movements  have  ceased.  It  may  be 
satisfactorily  combined  with  seclusion  behind  screens,  as 
carried  out  in  the  wards  of  a  hospital,  or  under  the  care  and 
supervision  of  a  nurse  in  private  practice. 

Abundance  of  nourishment,  mainly  milk  and  eggs,  should 
be  given.  If  the  malady  is  severe,  the  patient  may  have  to 
be  fed  by  hand,  or  even  by  means  of  a  tube  passed  through 
the  nose. 

Drugs  are  often  of  assistance  in  association  with  the  above. 
In  the  definitely  rheumatic  cases,  in  which  the  onset  of  the 
disease  has  been  preceded  or  accompanied  by  rheumatic  pains 
or  arthritis,  the  salicylates  or  aspirin  are  of  great  value.  In 
our  experience  aspirin  is  more  efficacious  than  the  salicylates, 
and  is  usually  well  borne.  It  should  be  given  in  doses  of  10 
to  15  grs.  every  four  hours  for  two  or  three  days,  and  then 
reduced  to  thrice  daily.  In  those  cases  without  obvious 
rheumatic  manifestations,  the  administration  of  arsenic 
(Fowler's  solution  in  doses  increasing  from  three  to  five,  ten 
or  more  drops  thrice  daily)  is  recommended.  The  long-con- 
tinued use  of  arsenic,  even  in  small  doses,  should  be  carefully 
watched,  as  it  is  known  to  have  induced  gastro-intestinal 
symptoms,  pigmentation  of  the  skin,  and  arsenical  paralysis, 
sometimes  of  an  intractable  character. 

Chloral  hydrate,  in  doses  sufficiently  large  to  induce  sleep, 
has  been  found  of  use  in  chronic  cases. 

In  the  severe  types  of  the  disease,  hydrobromate  of 
hyoscine  (gr.  —-,  to  ^)  given  hypodermically  is  of  much 
service.  In  these  cases  also  alcohol  may  act  as  a  most  efficient 
sedative. 

In  cases  with  much  psychical  disturbance  the  bromides 
are  of  especial  value. 

During  convalescence  tonic   treatment  by  means  of   cod- 


DISORDERS  OF  MOTION  507 

liver  oil  and  iron  are  essential.  A  tendency  to  tonsillitis  or 
enlargement  of  the  tonsils  should  also  be  attended  to,  as  the 
presence  of  hypertrophied  tonsils  favours  a  relapse  of  the 
symptoms. 


Chapter  III 
TETANY 

This  disease  is  characterised  by  the  occurrence  of  paroxys- 
mal or  continued  tonic  spasms,  bilateral  in  distribution, 
affecting  chiefly  the  muscles  of  the  extremities,  but  some- 
times also  those  of  the  trunk  and  face,  associated  with  a 
characteristic  excitability  of  the  nerves  and  muscles  to 
mechanical  and  electrical  stimulation. 

Etiology.  The  disease  is  more  especially  one  of  child- 
hood, and  boys  suffer  more  than  girls.  The  underlying  causes 
appear  to  be  debility,  and  toxic  poisoning  arising  from  gastric 
and  intestinal  disorder.  It  is  often  associated  with  rickets 
and  -  dilatation  of  the  stomach.  Amongst  adults  it  has 
been  observed  in  women  debilitated  during  pregnancy  or 
after  prolonged  lactation,  and  in  other  cases  has  followed 
directly  upon  the  removal  of  the  thyroid  gland.  An  acute 
epidemic  form  has  been  described  in  Austria  in  which  during 
the  spring  shoemakers  and  tailors  were  specially  affected. 
Other  cases  have  followed  on  poisoning  by  chloroform,  lead, 
and  ergot  of  rye. 

Symptoms.  The  earliest  sign  of  the  malady  is  numbness 
and  tingling  in  the  hands  and  feet,  followed  shortly  by 
the  sudden  onset  of  tonic  spasm  in  the  muscles  of  the 
extremities.  The  attitude  of  the  hands  and  feet  is  character- 
istic. The  fingers  are  flexed  at  the  metacarpo-phalangeal 
and  extended  at  the  interphalangeal  joints ;  the  thumb 
and  the  points  of  the  fingers  are  approximated,  and  the  hand 
assumes  a  cone-shaped  appearance.  The  wrists  are  flexed 
and  pronated,  the  elbows  flexed,  and  the  arms  adducted.  The 
feet  are  arched  and  inverted,  with  the  toes  pointed  and 
the  legs  extended  and  adducted  in  tonic  contraction.     More 


508  NERVOUS  DISEASES 

rarely  the  muscles  of  the  face  and  jaw  are  affected,  and 
sometimes  those  of  the  pharynx,  larynx,  and  tongue.  In 
rare  cases  the  trunk  muscles  may  also  be  involved. 

The  spasm  varies  in  intensity  and  duration.  It  may  last 
for  a  few  minutes  or  persist  for  several  days.  It  may  be 
overcome  by  force  or  resist  all  attempts  at  relaxation.  Its 
onset  is  sudden,  but  its  resolution  is  gradual.  It  may  at 
times  be  attended  by  pain  in  the  muscles. 

Several  characteristic  phenomena  are  present  during  an 
attack : — 

(a)  The  spasm  may  be  induced  by  pressure  on  a  nerve 
trunk  or  plexus. 

(/>)  Percussion  of  a  motor  nerve  may  cause  tetanic 
contraction  of  a  muscle. 

(c)  Percussion  or  electrical  stimulation  of  a  sensory  nerve 
may  induce  a  spasm. 

((/)  The  muscular  reaction  to  galvanism  is  of  a  tetanic 
character,  and  the  anodal  closing  contraction  is  greater  than 
the  cathodal.  No  objective  sensory  disturbance  is  present. 
The  deep  reflexes  are  brisk  and  the  plantar  response  is  of 
the  flexor  type. 

During  the  course  of  the  malady  the  temperature  is  raised 
and  the  pulse  accelerated,  and  oedema,  redness,  and  sweating 
of  the  hands  and  feet  may  be  observed. 

Prognosis.  Piecovery  usually  takes  place  when  the 
exciting  cause  has  been  removed.  Death  from  tetany  is  rare, 
but  may  occur  as  a  result  of  malnutrition  from  gastric  and 
intestinal  disorder. 

Treatment.  A  brisk  purge  should  be  administered  in 
all  cases,  and  attention  paid  to  the  diet.  In  cases  in  which 
gastric  dilatation  is  present,  lavage  of  the  stomach  should 
be  carried  out.  The  administration  of  intestinal  antiseptics, 
such  as  salol,  may  be  of  use.  During  the  attack,  complete 
rest,  with  bromides  and  chloral  internally,  are  of  value. 
In  severe  cases  it  may  be  necessary  to  give  inhalations  of 
chloroform  or  injections  of  morphia.  In  those  cases  directly 
due  to  removal  of  the  thyroid  gland,  extract  of  thyroid  may 
be  administered  with  advantage. 


DISORDERS  OF  MOTION  509 

Chapter  IV 
OCCUPATION   NEUROSES 

This  condition  is  a  local  disturbance  of  innervation  of  the 
muscles  required  in  the  performance  of  special  acts  or  move- 
ments necessary  for  the  carrying  out  of  certain  occupations. 

Although  the  action  of  the  muscles  is  impaired  mainly  in 
so  far  as  the  special  movements  are  concerned,  yet  in  the 
severe  types  of  the  disease  other  actions  of  the  affected  muscles 
may  be  interfered  with. 

Paralytic  conditions  associated  with  the  carrying  out  of 
certain  trades,  and  known  as  '  craft  palsies.'  have  also  been 
described.  These  differ  from  the  occupation  neuroses  by  the 
presence  of  muscular  atrophy  and  sensory  disturbances 
suggestive  of  a  localised  peripheral  neuritis. 

Etiology.  The  occupation  neuroses  occur  chiefly  in  those 
whose  profession,  or  trade,  requires  the  constant  use  and 
application  of  certain  muscles  of  the  hand  and  arm,  more 
rarely  of  the  lower  limb.  The  finer  the  movement  and  the 
more  persistently  it  is  applied  over  long  periods  of  time,  the 
more  readily  will  the  neuroses  develop  in  those  predisposed 
towards  them. 

They  are  found  in  persons  in  a  debilitated  state  of  health, 
and  principally  in  those  of  a  nervous  disposition,  who  are 
often  at  the  same  time  suffering  from  neurasthenia. 

They  are  more  often  observed  in  males  of  adult  age  than 
in  females,  and  they  are  not  unknown  in  children.  The 
occupations  in  which  they  most  commonly  occur  are  writing, 
sewing,  painting,  composing,  piano-  and  violin-playing,  and 
typewriting. 

Symptoms.  The  symptoms  may  be  described  according 
as  they  are  of  a  neuralgic  or  spasmodic  and  cramp-like 
character.  In  mild  cases  a  sense  of  discomfort,  of  aching,  or 
of  a  readiness  to  fatigue,  rather  than  actual  pain  may  be  the 
only  symptom.  In  the  more  acute  types  definite  pain,  rarely 
however  accompanied  by  tenderness  along  the  nerves,  may  be 
present.  A  prolonged  and  persistent  effort  over  many  hours 
at  writing  may  lead  to  a  sense  of  fatigue  in  the  hand  and  arm, 
which  should  not  be  regarded  as  a  definite  neurosis,  for  a 


510  NERVOUS  DISEASES 

short  rest  will  relieve  the  symptom.  Should,  however,  the 
strain  thus  induced  be  continued  daily  over  many  months 
or  years  in  predisposed  persons,  a  definite  type  of  occupation 
cramp  or  neuralgia  will  eventually  be  developed. 

A  survey  of  the  symptoms  found  in  the  several  forms  of 
occupation  cramp  reveals  the  fact  that  the  muscles  primarily 
affected  are  those  subjected  to  severe  and  prolonged  strain,  as 
in  holding  an  object  such  as  a  pen,  or  in  forcible  contraction, 
as  occurs  in  the  piano-player  or  the  violinist.  Prolonged  and 
often-repeated  muscular  effort  of  any  nature  is  a  frequent 
cause  of  the  neurosis. 

The  spasmodic  or  cramp-like  symptoms  may  be  described 
in  conjunction  with  the  muscular  effort  required  to  carry  out 
the  action. 

(a)  Occupations  requiring  the  use  of  the  prehensile  muscles 
of  the  hand,  such  as  writing,  sewing,  painting,  composing. 

The  commonest  neurosis  of  this  class  is  writer's  cramp, 
which  may  be  taken  as  a  type  of  the  affection.  The  spasm 
affects  the  flexors  of  the  thumb  and  index  finger.  The  pen  is 
forcibly  gripped  for  a  time,  but  eventually  slips  from  the 
hand ;  or  the  spasm  may  lead  to  an  irregular  and  misdirected 
use  of  the  pen,  the  point  of  which  catches  on  the  paper.  The 
spasm  may  gradually  overflow  from  the  hand,  so  as  to  affect 
the  extensors,  supinators,  and  pronators  of  the  wrist.  The 
writing  is  jerky  and  irregular,  and  the  paper  shows  splutters 
of  ink  where  the  point  of  the  pen  has  caught.  It  is  accom- 
panied by  pain  in  the  ball  of  the  thumb,  in  the  index  finger, 
and  about  the  wrist. 

In  tailors  and  seamstresses  the  thumb  and  index  finger 
are  those  mainly  affected.  In  dairymaids  the  cramp  is  in 
the  flexors  and  extensors  of  the  wrist  and  fingers. 

(b)  Occupations  requiring  forcible,  but  not  necessarily 
continuous  muscular  efforts,  as  in  piano-  and  violin-playing, 
typewriting,  and  flute-playing. 

In  pianists  the  cramp  takes  the  form  of  a  spasmodic 
raising  of  the  fingers  from  off  the  keys,  or  of  their  forcible 
retention  upon  them.  The  strain  is  mainly  upon  the  extensor 
muscles  of  the  forearm,  and  the  pain  is  occasionally  severe, 
and  affects  the  upper  arm  and  shoulder  as  well  as  the  forearm 
and  wrist. 

In   violinists    and    'cellists  the  strain  is  chiefly  upon  the 


DISOKDEKS  OF  MOTION  511 

left  or  string  hand,  the  lingers  of  which  press  upon  the 
strings,  and  in  'cellists  particularly  upon  the  abductor  and 
opponens  pollicis  (Poore).1  The  cramp  is  usually  accompanied 
by  pain  in  the  arm  and  shoulder.  The  right  or  bow  hand 
may  also  be  the  seat  of  cramp,  owing  to  the  strain  upon  the 
prehensors  of  the  hand,  but  this  is  less  common. 

(c)  Occupations  requiring  frequently  repeated  muscular 
efforts  accompanied  by  strain.  Examples  of  this  are  seen  in 
blacksmiths,  in  whom  the  upper  arm  and  shoulder  muscles, 
especially  the  triceps  and  the  deltoid,  are  implicated,  and 
hammermen,  where  a  difficulty  occurs  in  directing  the  arm 
which  holds  the  hammer.  In  a  case  of  treadler's  cramp 
described  by  Rivers,  the  glutei  and  the  hamstring  muscles  of 
the  right  leg  were  affected.  Tiptoe  dancers  are  notoriously 
prone  to  spasm  of  the  muscles  of  the  calves  of  the  legs. 

Many  other  occupations  from  time  to  time  occasionally 
present  a  case  of  occupation  neurosis.  We  have  seen  spasm 
of  the  tongue  in  a  cornet-player  ;  spasm  of  the  orbicularis 
palpebrarum  has  been  recorded  in  watchmakers  ;  and  miners' 
nystagmus  is  a  well-recognised  condition  in  those  who  work 
in  a  recumbent  posture  with  the  eyes  turned  upwards  to 
one  side. 

The  general  symptoms  of  the  occupation  neuroses  are  those 
of  neurasthenia,  or  of  any  associated  disorder  which  may 
be  present.  Some  degree  of  tremor  of  the  hands  is  an 
invariable  accompaniment,  and  is  usually  more  marked  in 
the  affected  limb.      The  deep  reflexes  are  brisk. 

Differential  diagnosis.  Most  cases  present  no  difficulty, 
but  symptoms  resembling  those  of  an  occupation  neurosis  may 
arise,  either  in  the  early  stages  of  organic  nervous  disease  or 
from  a  local  condition.  It  is  therefore  necessary  to  exclude 
all  such  conditions.  The  following  are  the  disorders  in  which 
difficulty  in  diagnosis  may  arise : — 

Paralysis  agitans.  Tremor,  weakness,  and  spasticity  of 
the  thumb  and  finger  muscles  give  rise  to  difficulty  and 
cramp  in  holding  the  pen. 

Brachial  neuralgia  resembles  in  many  respects  the  neu- 
ralgic form  of  the  occupation  neuroses.  Although  often 
associated  with  symptoms  of  neurasthenia,  this  variety  of  the 

1  Poore,  AllbuWs  System  of  Medicine,  vol.  viii. 


512  NERVOUS  DISEASES 

malady  is  not  accompanied  by  the  spasmodic  contraction  of 
the  muscles  found  in  the  genuine  cramp,  but  presents  the 
neuralgic  symptoms  in  a  marked  degree. 

Partial  paralyses  of  the  peripheral  nerves  of  the  arm  have 
been  known,  also,  to  give  rise  to  symptoms  having  a  resem- 
blance to  the  occupation  palsies. 

Articular  affections  such  as  rheumatoid  arthritis,  teno- 
synovitis of  the  fingers  or  wrist,  are  other  conditions  which 
may  interfere  with  movements  of  the  hand. 

There  is  an  hysterical  or  psychical  occupation  neurosis,  in 
which  a  fear  of  inducing  the  disorder  may  interfere  with 
writing,  perhaps  owing  to  the  patient  having  seen  it  in 
others,  or  of  having  heard  or  read  of  its  effects.  The  appre- 
hension may  be  so  vivid  as  to  become  almost  a  fixed  idea,  and 
produce  a  state  of  mind  in  which  writing  becomes  impossible. 
This  is  a  rare  condition. 

Prognosis.  The  spasmodic  forms  of  occupation  neuroses 
are  prolonged  and  intractable  conditions.  By  aid  of  complete 
rest  considerable  alleviation  may  be  obtained,  but  a  return 
to  work,  even  where  the  malady  seems  to  have  been  cured, 
is  frequently  followed  by  a  relapse.  In  the  neuralgic  cases, 
especially  those  complicated  with  neurasthenia,  recovery  is 
rapid,  but  relapses  are  liable  to  occur. 

Treatment.  The  first  essential  lies  in  desisting  entirely 
from  the  occupation  which  has  given  rise  to  the  neurosis. 
In  some  cases,  more  especially  in  writers,  the  condition 
arises  from  faulty  methods.  Such  should,  on  resuming  work, 
be  corrected.  During  the  period  immediately  following  the 
cessation  of  work,  local  treatment  to  the  affected  limb  or 
limbs,  especially  massage  of  the  muscles  and  carefully  selected 
Swedish  exercises,  should  be  prescribed.  Electrical  treatment, 
particularly  galvanism,  may  be  adopted  with  advantage.  In 
the  obviously  neurasthenic  cases  a  complete  or  modified  rest 
cure  has  been  found  of  great  benefit.  In  cases  complicated 
with  brachial  neuralgia,  the  arm  requires  to  be  rested  in  a 
sling,  and  the  other  methods  of  treatment  for  this  condition 
adopted. 

Drugs  may  be  prescribed  as  required.  Tonics  are  of  use 
in  the  later  stages,  but  sedatives  may  be  needed  in  the 
earlier. 

Treatment  should  be   continued  for    a  prolonged   period. 


DISORDERS  OF  MOTION  513 

Even  after  a  year's  rest  a  return  to  work  may  induce  a 
relapse  of  the  symptoms.  Some  cases  never  entirely  recover  : 
in  which  event  it  is  necessary  for  the  patient  to  take  up 
another  occupation. 

Craft  Palsies 

Atrophy  of  the  small  muscles  of  the  hand  occurs  in  certain 
occupations  requiring  long-continued  over-exertion  of  certain 
muscles  (Oppenheim).  This  atrophy  is  partly  due  to  over- 
exertion and  partly  to  pressure  upon  the  nerves. 

Craft  palsies  occur  most  commonly  in  platers,  filers,  lock- 
smiths, rowers,  glass -workers,  and  cigar -rollers.  Similar 
affections  have  been  found  in  the  lower  limbs  in  potato- 
pickers,  seed-sowers,  and  others. 

In  addition  to  muscular  atrophy,  paresthesia,  numbness, 
and  pains  are  present,  indicating  pressure  upon  the  peripheral 
nerves.  Alcoholism  appears  to  favour  their  occurrence,  and 
other  toxic  conditions -such  as  lead-poisoning,  influenza, 
and  general  states  of  debility — may  also  act  as  predisposing 
factors. 

The  prognosis  is  on  the  whole  favourable,  and  the  treatment 
consists  in  complete  rest  and  galvano-faradic  stimulation  of 
the  atrophied  muscles,  with  tonics  internally. 


TETANOID  CHOREA 

This  disease  has  derived  its  name  from  the  appearances 
which  were  observed  by  Gowers  in  two  cases  described  in  1888.1 

The  disease  is  probably  due  to  morbid  changes  induced  by 
inherited  syphilis,  as  Homen  suggested  in  1890.  The  changes 
found  after  death  have  been  symmetrical  softening  of  both 
lenticular  nuclei,  sometimes  with  thickening  of  the  membranes 
and  of  the  skull.  These  changes  in  the  nervous  system  are 
associated  with  cirrhosis  of  the  liver. 

The  leading  features  are :  its  onset  during  apparent  health, 
the  progressive  character  of  the  symptoms,  its  occurrence  in 
families,  and  its  association  with  cirrhosis  of  the  liver.  It 
affects  both  males  and  females,  and  the  symptoms  appear  in 
late  childhood  or  early  adult  life. 

1  Gowers,  Diseases  of  the  Nervous  System,  vol.  ii,  1S88. 


514  NERVOUS  DISEASES 

The  symptoms  consist  of  slow,  continued  tetanoid  spasms 
of  the  limbs,  sometimes  involving  the  masseters  and  facial 
muscles.  Difficulty  in  protruding  the  tongue,  or  in  closing 
the  jaws,  owing  to  pain  has  often  been  observed,  and  gives 
rise  to  dysarthria  and  difficulty  in  swallowing.  These  move- 
ments tend  to  become  less  marked  or  less  constant,  while 
rigidity  develops  along  with  progressive  mental  impairment 
and  general  emaciation. 

Death  may  occur  within  a  few  months,  or  life  may  be 
prolonged  over  several  years. 


Part  XIV 
MIGRAINE   AND   PERIODIC   HEADACHE 

Migraine  and  periodic  headache  are  described  together,  as 
they  appear  to  be  essentially  of  the  same  nature,  although  the 
phenomena  presented  in  any  given  case  may  vary  from  the 
typical  migraine  seizures  with  their  peculiar  visual  symptoms 
and  gastric  disturbances,  to  a  localised  headache  accompanied 
or  succeeded  by  nausea. 

The  characteristic  features  are  the  periodicity  of  the 
seizures,  their  persistence  throughout  the  whole  of  early  adult 
life,  their  tendency  to  disappear  or  lessen  towards  the  end 
of  sexual  life,  their  essentially  hereditary  character,  and  the 
absence  of  any  obvious  or  well  ascertained  cause  for  their 
occurrence. 

Etiology.  Periodic  headaches  may  commence  at  any  age, 
but  more  commonly  during  childhood  and  puberty.  Cases 
on  the  other  hand  are  observed  in  which  the  attacks  of 
headache  have  not  appeared  until  adult  life.  Although  it  is 
usual  for  these  headaches  to  diminish  both  in  severity  and 
frequency  at  or  after  the  climacteric,  it  is  found  that  some 
increase  of  the  symptoms  may  date  from  this  epoch  in  a 
number  of  cases. 

Women  are  probably  more  liable  to  periodic  headache 
than  men. 

Heredity  plays  an  important  part  in  the  causation  of 
migraine.  It  is  a  disease  which  runs  in  families.  Many 
members  of  the  same  family  are  subject  to  one  or  other  form 
of  paroxysmal  headache.  The  type  of  headache  is  not  always 
the  same.  A  mother,  who  suffers  from  typical  migraine,  may 
have  one  or  more  children  who  are  subject  to  periodic  headache 
without  the  visual  symptoms  ;  or  paroxysmal  headache  may 
occur   as   the  type  of  neurosis  in  one  member  of  a  family, 

515  33  * 


516  NERVOUS  DISEASES 

whose  other  members  suffer  from  functional  disorders  of 
which,  hysteria,  hay-fever  and  '  nervousness '  may  be  mani- 
festations. 

There  exists,  moreover,  a  relation  between  paroxysmal 
headache  and  epilepsy.  Cases  are  constantly  met  with  in 
which  periodic  headache  in  one  of  the  parents  may  be 
associated  with  epilepsy  in  the  offspring.  It  has  also  been 
observed  that  the  periodic  headaches  of  early  life  may  be 
replaced  bj7  epileptic  fits  in  the  later  years,  and  the  coexistence 
of  periodic  headache  and  epilepsy  in  the  same  person  is  a 
feature  occasionally  observed. 

No  constant  exciting  cause  has  been  determined.  Of  those 
which  favour  the  onset,  overwork,  worry,  anxiety,  fatigue 
and  mental  excitement  are  the  most  common.  Digestive 
disturbances,  anaemia,  and  debility  are  also  frequent.  The 
menstrual  periods  are  especially  liable  to  determine  headaches 
in  those  subject  to  them.  Errors  of  refraction  are  a  not 
uncommon  association.  Stress  has  been  laid  upon  the 
influence  of  toxic  conditions— more  especially  of  gout  and 
uric  acid. 

Symptoms.  The  symptoms  of  this  disease  consist  of 
attacks  of  headache,  which  may  or  may  not  be  preceded  by 
prodromal  symptoms,  and  may  or  may  not  be  associated  with 
visual  phenomena.  They  are  usually  unilateral,  and  are 
accompanied  or  succeeded  by  nausea,  retching,  or  vomiting. 

Although  some  persons  have  no  warning,  others  are  usually 
made  aware  of  their  approach  by  premonitory  symptoms:  of 
which  a  feeling  of  coldness  in  the  extremities,  excessive  fatigue, 
a  'heady'  sensation,  restlessness,  and  even  insomnia  the  night 
before,  may  be  mentioned. 

The  headache  may  come  on  during  the  early  morning, 
waking  the  patient  from  sleep;  or  during  the  day,  when  its 
onset  is  preceded  by  a  definite  aura.  In  some  cases  the  aura 
consists  of  loss  or  perversion  of  the  visual  functions.  In  other 
cases  the  aura  is  in  the  form  of  numbness,  paresthesia,  or 
perversions  of  common  sensibility  in  the  limbs,  face,  or  tongue. 
If  on  the  right  side,  a  paraphasic  affection  of  speech  may 
accompany  it.  With  the  cessation  of  the  aura  the  headache 
comes  on. 

The  headache  varies  much  in  character.  Sometimes  it  is 
of  a  boring,  penetrating  nature ;  at  other  times  it  is  described 


MIGRAINE  AND  PERIODIC  HEADACHE  517 

as  pulsating  or  throbbing,  or  it  is  said  that  the  head  feels  as 
if  gripped  in  a  vice. 

The  position  of  the  headache  also  varies.  It  is  usually 
frontal,  or  referred  to  the  orbit,  forehead,  or  fronto-temporal 
region.  In  other  cases  it  is  referred  to  the  root  of  the  nose, 
or  supra-orbital  margin.  It  may  remain  limited  to  these 
regions;  or,  as  already  described,  it  may  radiate  over  the 
vertex  to  the  occipital  pole.  In  rarer  cases  it  commences  at 
the  back  of  the  head;  and  tends  to  diffuse  itself  towards  the 
vertex  and  temples.  Its  presence  gives  rise  to  great  intoler- 
ance of  light  and  sound.  The  victims  of  these  attacks  lie 
down  in  a  darkened  room  and  shut  themselves  off  from  all 
noise  or  communication  with  others.  There  is  also  a  profound 
aversion  to  food  during  the  persistence  of  the  attack. 

It  has  been  urged  as  a  point  of  differential  diagnosis 
between  epilepsy  and  migraine,  that,  in  the  latter  condition, 
mental  symptoms  following  the  paroxysm  do  not  occur.  In 
many  cases  this  is  so;  but  in  others  — owing  mainly  to  the 
intensity  of  the  pain— hysterical  manifestations,  stupor,  and 
delirium  have  been  described.  On  the  other  hand,  notwith- 
standing the  frequency  of  the  attacks  of  migrainous  headache 
or  the  persistence  of  the  malady  over  many  years,  mental 
impairment  of  a  permanent  character  is  never  observed. 

Visual  symptoms  are  found  in  about  half  the  cases  of 
periodic  headache,  and  when  present  always  form  the  aura,  or 
warning  of  the  impending  seizure.  They  vary  greatly  in 
different  cases,  but  those  most  commonly  described,  fall  into 
one  or  other  of  the  following  types  :  — 

(a)  Central  scotoma,  or  defect  in  or  abolition  of  the  central 
vision.  This  spot  enlarges  from  the  centre  to  the  periphery, 
and  as  it  expands  laterally  clears  up  centrally.  In  the  central 
dim  area  spectral  phenomena  may  appear — such  as  flickering 
lights,  or  a  central  bright  spot,  or  the  edge  of  the  spot  takes 
on  a  zigzag  shape,  or  '  fortification  spectrum.' 

(b)  Hemianopsia,,  or  blindness  of  the  corresponding  half- 
fields  in  both  eyes.  This  usually  begins  as  a  small  area,  and 
expands  until  the  half-field  is  completely  obliterated. 

(c)  Indefinite  symptoms— such  as  coloured  lights,  objects 
in  constant  vibration  comparable  to  what  is  seen  in  cinemato- 
graphic pictures,  sparks,  or  flashes  of  light. 

Sensory  symptoms  when  present  are  referred  to  the  upper 


518  NERVOUS  DISEASES 

limbs,  the  face,  tongue,  and  lips,  and  only  rarely  to  the  legs. 
They  are  generally  unilateral  occurring  on  the  hemianopic 
side.  They  consist  of  tingling  and  paresthesia  which  pass 
up  the  limb  into  the  face  and  mouth;  or  the  arm  only,  or 
the  mouth  only,  may  be  affected.  Following  the  subjective 
sensations  come  numbness  and  sometimes  impairment 
or  loss  of  sensation.  They  may  be  succeeded  by  slight 
and  temporary  loss  of  power  in  the  arm. 

If  aphasia  is  present  it  is  usually  of  the  nature  of 
paraphasia,  or  inability  to  find  the  word,  or  to  use  the  wrong 
word ;  only  rarely  has  complete  motor  aphasia  been  observed. 

Types  of  migraine.  Attacks  of  migraine  vary  in  different 
persons,  but  in  all  a  somewhat  similar  sequence  occurs,  which 
may  be  best  described  by  referring  to  some  illustrative  cases. 

(a)  The  attack  may  be  preceded  by  a  feeling  of  faintness 
and  a  craving  for  food.  About  an  hour  later  the  attack  itself 
commences  with  a  feeling  of  numbness  and  deadness  in  the 
tip  of  the  tongue  and  in  the  fingers  of  the  right  hand,  some- 
times also  of  the  left.  This  is  followed  by  a  feeling  of  coldness 
and  general  pallor.  In  from  five  to  ten  minutes  the  numbness 
spreads  to  the  lips,  mouth,  and  face,  and  speech  becomes 
paraphasia.  About  this  time  vision  becomes  affected,  with 
loss  first  of  the  central  field,  rapidly  followed  by  right-sided 
hemianopsia,  occasionally,  but  rarely,  accompanied  by  zigzag 
lights  in  the  right  temporal  field.  About  twenty  minutes  from 
the  onset,  the  subjective  visual  and  sensory  symptoms  begin  to 
fade  and  are  replaced  by  a  dull,  persistent,  boring  pain  strictly 
limited  above  the  left  eyebrow;  perspiration,  nausea,  and 
vomiting.  The  headache  increases  in  intensity,  and  the 
general  sensation  of  cold  assumes  a  more  intense  and  throbbing 
character ;  the  pallor  then  passes  off,  and  the  coldness  gives 
place  to  a  sensation  of  warmth.  The  headache  gradually 
lessens  and  disappears  in  from  twelve  to  twenty-four  hours, 
leaving  a  feeling  of  great  prostration  behind  it. 

(b)  The  first  sign  of  the  impending  attack  is  a  sudden 
temporary  defect  of  central  vision,  in  which  the  figures,  for 
example,  of  a  watch  are  obliterated  or  rendered  indistinct. 
This  is  followed  after  a  few  minutes  by  the  appearance  of 
a  flickering  patch  of  pure  white  light,  as  if  many  thin 
intersecting  lines  were  dancing  irregularly  to  and  fro.  This 
sensation  is  always  one-sided,  and  the  headache  which  follows 


MIGRAINE  AND  PERIODIC  HEADACHE  519 

is  usually,  if  not  invariably,  on  the  opposite  side  to  that  on 
which  the  light  appears.  After  some  fifteen  or  twenty  minutes 
the  flickering  light  disappears,  and  is  followed  by  a  localised 
headache— always  frontal  and  unilateral.  This  usually  lasts 
for  some  hours,  and  is  accompanied  by  flatulence,  eructations, 
nausea,  and  sometimes  by  fits  of  violent  retching  and  vomiting. 
After  the  headache  has  passed  away,  there  is  commonly  a  free 
flow  of  pale  or  colourless  urine. 

(c)  The  attack  begins  with  a  warning  sensation  of  flushing 
of  the  face  and  a  feeling  of  coldness  in  the  extremities. 
Severe  pain  of  a  throbbing  or  pulsating  character  then  starts 
in  the  region  of  the  temples,  sometimes  on  the  right,  sometimes 
on  the  left  side.  The  pain  always  remains  for  a  time  on 
the  side  upon  which  it  starts.  If  the  headache  is  very 
severe  it  may  spread,  later  on,  over  the  head.  Na'usea,  severe 
retching  or  vomiting,  persist  throughout  the  attack. 

During  the  attack  the  patient  passes  into  a  hysterical  state 
from  the  intensity  of  the  pain.  After  this  is  over  she  is 
prostrated  and  exhausted  for  one  or  two  days. 

(d)  A  definite  pain  in  the  head  begins  to  come  on  in  the 
early  hours  of  the  morning,  but  finally  settles  down  in  the 
region  of  the  right  eye,  forehead,  and  temple.  It  does  not 
radiate  to  the  back  of  the  head,  but  occasionally  passes 
through  to  the  right  side  of  the  neck  or  shoulder.  After  the 
headache  has  persisted  for  about  six  hours,  retching  and 
vomiting  commence  and  continue  along  with  the  headache, 
which  rarely  ceases  until  the  evening  of  the  following  day. 
After  the  attack  is  over  there  is  sometimes  difficulty  in  raising 
the  right  upper  lid. 

(e)  The  attack  begins  with  pain  usually  in  the  left  eye, 
which  extends  into  and  down  the  nose.  It  then  passes  along 
the  affected  side  of  the  head  to  the  occipital  region.  During 
the  persistence  of  the  headache,  retching  and  vomiting  come 
on  and  last  for  about  twenty-four  hours. 

Course  and  prognosis.  The  attacks  of  headache  vary 
much  in  frequency  and  duration.  They  may  be  as  frequent  as 
two  or  three  per  week,  or  as  infrequent  as  two  or  three  yearly. 
In  severe  cases  they  may  be  counted  by  the  week,  fortnight,  or 
month.  They  may  come  on  at  any  time,  but  usually  in  the 
early  morning  or  during  the  forenoon.  The  experience  of 
patients  varies  as  regards  the  duration  of  the  seizure.     The 


520  NERVOUS  DISEASES 

common  duration  of  an  average  seizure  is  from  six  to  eight  or 
ten  hours  ;  in  niany  cases  the  headache  persists  for  twenty- 
four  hours  or  more,  and  some  attacks  of  headache  do  not 
entirely  abate  for  two  or  three  days  or  longer. 

The  tendency  is  towards  an  amelioration  of  the  severity, 
and  a  diminution  of  the  frequency  of  the  attacks  as  age 
advances.  This  is  not  invariably  the  case.  Some  of  the 
most  severe  and  prolonged  attacks  of  paroxysmal  headache 
have  been  seen  in  women,  who  have  passed  the  climacteric. 
A  considerable  number  of  female  patients  find  an  increase  in 
the  severity  and  frequency  of  the  headaches  some  years 
before  the  climacteric  is  reached. 

Treatment.  All  likely  excitants  of  the  attack,  of  which 
the  more  important  are  fatigue,  mental  and  bodily  stress  and 
strain,  overwork,  excitement,  and  late  hours,  should  be  avoided. 
Indiscretions  in  diet,  especially  towards  the  end  of  a  spell  of 
freedom  from  attacks,  should  be  avoided.  Errors  of  refraction 
should  be  searched  for  in  all  cases  in  young  people,  and,  if 
necessary,  corrected.  The  debilitating  influences  of  anaemia, 
influenza,  and  other  toxic  conditions  should  be  treated  along 
general  lines.  It  seems,  however,  impossible  to  prevent  an 
occasional  attack,  even  on  the  part  of  those  persons  who  have 
carefully  and  intelligently  studied  their  malady. 

Acting  on  the  theory  that  in  many  cases  the  attacks  are 
attributable  to  an  excess  of  uric  acid,  xanthin,  or  hypoxanthin, 
mainly  ingested  with  certain  articles  of  food,  the  purin  free 
diet,  suggested  by  Haig,  has  been  recommended  and  occasion- 
ally found  beneficial  in  reducing  the  number  and  severity  of 
the  attacks,  and  in  some  instances  even  arresting  them 
altogether.  The  cases  successfully  treated  in  this  way  have 
been  associated  with  an  increase  of  the  arterial  tension  before 
the  attack. 

On  account  of  the  persistent  character  of  the  malady  and 
tendency  for  the  attacks  of  headache  to  recur  frequently, 
many  drugs  have  been  recommended,  and  tried — some  with  a 
view  to  diminish  or  arrest  the  attacks,  and  others  to  abort, 
modify,  or  subdue  the  seizures  as  they  arise. 

Amongst  the  former,  the  bromides,  administered  as 
described  under  epilepsy,  alone  or  in  combination  with 
phenacetin  and  caffeine,  have  been  of  service  in  many  cases. 
The  tinctures  of  gelsemium  or  of  belladonna  have  been  found 


MIGRAINE  AND  PERIODIC  HEADACHE         52] 

useful  auxiliaries  to  the  bromides.  The  prolonged  adminis- 
tration of  the  valerianates  of  zinc  and  iron,  with  or  without 
the  addition  of  small  doses  of  cannabis  indica,  has  also  been 
found  of  advantage  in  cases  complicated  with  anaemia,  or 
in  which  the  bromides  are  badly  taken  or  inconvenient. 
Nitro-glycerine  has  been  strongly  recommended  in  cases 
characterised  by  high  arterial  tension. 

As  remedies  for  the  temporary  alleviation  of  the  attacks, 
all  the  synthetical  analgesics  may  be  tried — antipyrin, 
phenacetin,  exalgin,  phenalgin,  aspirin,  &c.  One  or  other  of 
them,  alone  or  in  combination,  prescribed  as  a  draught 
during  the  premonitory  stage  and  repeated  in  an  hour  or 
two,  will  probably  be  useful  in  the  majority  of  cases,  although 
now  and  again  a  case  will  occur  in  which  they  prove  to  be 
of  little  service. 

A  useful  remedy  is  the  administration  of  phenacetin 
(5  grs.)  and  caffein  (2  grs.)  as  soon  as  the  attack  commences, 
followed  by  liq.  strychnine  (5  ms.)  when  the  headache  begins 
to  lessen. 


Part   XV 

HYSTERIA 

Hysteria  is  a  ps}Tchical  disorder,  arising  from  a  functional 
disturbance  of  the  cerebral  cortex.  Its  fundamental  charac- 
teristic is  a  dissociation  or  severance  of  some  mental 
processes  from  the  main  personal  consciousness  (Janet).1  This 
dissociation  is  the  basis  of  both  the  temporary  and  the  persis- 
tent phenomena  of  the  disease,  and  shows  the  common  origin 
of  such  diverse  conditions  as  the  hysterical  fit,  dual  personality, 
anaesthesia,  and  paralysis.  In  the  hysterical  paroxysm, 
the  patient  passes  through  a  phase  of  movements  and  actions 
of  which  there  is  little  or  no  subsequent  recollection ;  and  in 
the  paralyses,  there  is  loss  of  consciousness  of  the  existence 
of  the  affected  limb  or  side  of  the  body. 

Etiology 

Hj^steria  is  commonest  during  puberty  and  early  adoles- 
cence, but  is  not  unknown  in  childhood.  It  may  disappear 
during  adult  life,  but  is  liable  to  recur  in  persons  of  neuro- 
pathic disposition.  The  female  sex  is  more  often  affected  than 
the  male,  especially  in  later  life.  It  would  seem  to  have  a 
special  proclivity  to  affect  the  Latin,  Slavonic,  and  Jewish 
races. 

It  occurs  in  all  social  grades,  being  as  frequently  found  in 
hospital  as  in  private  practice. 

Heredity  plays  an  important  part  in  the  production  of  the 
disease.  The  neuropathic  disposition  is  shown  by  the 
occurrence  of  hysteria,  epilepsy,  insanity,  the  tics,  alcoholism, 
and  '  nervousness  '  in  other  members  of  the  family. 

1  Janet,  The  Major  Symptoms  of  Hysteria,  1007- 
522 


HYSTERIA  52:5 

Hysteria  may  be  excited  by  all  forms  of  emotional  dis- 
turbance-shock, fright,  worry,  or  grief  ;  by  prolonged 
ill-health— ansemia,  pelvic  disorders,  and  other  physical 
factors  of  a  like  kind.  Traumatism  is  a  common  cause 
of  hysteria,  which  is  attributable  rather  to  the  psychical 
conditions  attendant  upon  an  accident  than  to  any  bodily 
injury. 

Symptomatology 

Hysteria  is  characterised  by  a  variety  of  symptoms, 
differing  materially  in  type,  persistence,  and  distribution,  but 
all  attributable  to  the  same  fundamental  psychical  state. 

As  regards  type,  some  of  the  symptoms  —  such  as  paralysis 
-denote  a  loss  of  function;  while  others  — such  as  spasms, 
agitations,  and  tremors  -  indicate  an  excess  of  function.  When 
considered  from  the  standpoint  of  persistence,  some  are 
transitory  — such  as  the  convulsions  and  the  automatisms— and 
to  them  the  term  '  accidents '  of  hysteria  has  been  applied. 
Others  are  more  enduring,  and  on  account  of  their  persistence 
have  been  named  the  '  stigmata '  of  hysteria ;  these  are  the 
anaesthesias,  paralyses,  and  contractures. 

Hysterical  temperament.  The  principal  feature  of  the 
hysterical  temperament  is  an  undue  nervousness  and  sus- 
ceptibility to  both  physical  and  psychical  impressions.  On 
this  account  mainly,  normal  or  everyday  phenomena  are  not 
only  misinterpreted,  but  are  sometimes  so  exaggerated  as  to 
assume  an  altogether  disproportionate  significance. 

Minor  illustrations  of  this  state  are  seen  in  the  starts  or 
jumps  which  these  patients  exhibit  when  a  door  is  slammed, 
or  when  any  sudden  action  or  movement  occurs ;  or,  in  the 
psychical  sphere,  in  the  taking  to  heart  a  word  or  phrase 
spoken  in  jest  or  uttered  without  any  unkind  intent. 
Attacks  of  so-called  'hysterics' — such  as  screaming -which 
may  accompany  an  unusual  sight,  such  as  a  serious  accident, 
may  be  regarded  as  a  more  exaggerated  form  of  the  same 
condition. 

Another  feature  of  the  hysterical  temperament  is  suscepti- 
bility to  hypnotism  and  auto-suggestion. 

Hysterical  persons  also  may  possess  many  of  the  tempera- 
mental disabilities  common  to  neuropathic  subjects— such  as 
abnormalities  of  emotion  and  of  will  power.     Feebleness  of  will, 


524  NERVOUS  DISEASES 

indecision,  obstinacy,  and  doubt  are  often  common  accompani- 
ments of  the  hysterical  state.  Although  the  intelligence  is 
good  and  the  memory  normal,  judgment  is  often  weak  and 
rasily  iiitliiciiced  by  temporary  emotions. 

Mental  state.  The  basis  of  the  mental  state  in  hysteria 
consists  of  a  dissociation,  or  disparity  of  consciousness, 
whereby  certain  phases  of  consciousness  may  be  lost  or  per- 
verted. The  most  characteristic  instance  of  dissociation  is 
seen  in  the  condition  known  as  double  consciousness,  or  dual 
personality.  In  this  manifestation  of  hysteria  two  entirely 
separate  states  of  activity  are  found— one,  however,  being  the 
dominant  state.  In  one  of  these  phases  the  patient  is  her 
natural  self  in  thought,  word,  and  deed ;  but  in  the  other  she 
exhibits  a  different  and  sometimes  antagonistic  personality. 
There  is  no  conscious  recollection  of  what  has  occurred  in  one 
state  when  in  the  alternate  condition,  although  the  memory 
of  the  somnambulistic  phase  may  be  revived  by  hypnotic 
suggestion. 

Lesser  degrees  of  dissociation  are  illustrated  in  those 
amnesic  phases  in  which  portions  of  the  previous  existence 
of  the  patient  may  be  entirely  blotted  out  and  forgotten. 

In  cases  of  disparity  or  inequality  of  consciousness,  the 
phenomena  of  dissociation  are  less  extensive  or  less  complete. 
For  example,  there  may  be  a  failure  of  memory  confined  to 
certain  events  or  certain  persons  ;  or  there  may  exist  a  want 
of  conscious  appreciation  of  a  portion  of  the  body.  Thus,  one 
of  our  patients,  suffering  from  hysterical  paralysis  of  the  right 
arm  had  no  conscious  perception  of  her  arm,  and  stated  that 
she  felt  as  if  the  arm  had  been  'lopped  off'  at  the  shoulder 
joint. 

Hysterical  Paroxysms 

These  are  of  several  kinds  and  vary  both  in  intensity, 
character,  and  frequency.    They  are  : 

1.  Minor  Hysteria  ('Hysterics'). 

2.  Major  Hysteria. 

3.  '  Hystero-epilepsy.' 

4.  Automatic  phenomena  and  dual  personality. 

5.  Hysterical  stupor. 

1.  The  minor  hysterical  attack  may  follow  an  emotional 


HYSTERIA  525 

shock,  or  occur  without  any  obvious  cause.  It  commences 
with  a  sensation  of  palpitation,  or  of  a  rising  from  the  stomach, 
which  is  followed  by  a  sensation  of  a  lump  in  the  throat 
(globus  hystericus).  Repeated  acts  of  swallowing  are  made 
with  the  object  of  removing  the  sensation  of  the  lump.  A 
feeling  of  suffocation  ensues,  the  patient  tears  at  the  neck-band 
of  her  dress,  or  throws  herself  into  a  chair,  with  starting  eyes, 
in  a  state  of  great  agitation  and  distress.  She  pants  for 
breath  and  calls  for  help.  She  may  tear  her  hair,  or  wring 
her  hands,  or  she  may  swoon  or  break  out  into  uncontrolled 
laughter  or  weeping.  Recovery  gradually  ensues  with  eruc- 
tations of  wind,  especially  when  much  globus  has  been  present. 
A  large  quantity  of  pale  limpid  urine  is  usually  passed  after 
the  attack,  which  is  followed  by  considerable  exhaustion.  Any 
one  of  the  main  constituents  of  the  attack  may  form  the  sole 
feature  of  an  individual  seizure. 

2.  The  major  hysterical  attack.  The  seizure  may  be 
preceded  by  an  aura  of  pain  in  the  region  of  the  ovaries  or 
stomach  ;  or  by  the  globus  hystericus,  palpitation,  general 
excitement,  dizziness,  or  sensations  —usually  bilateral— referred 
to  the  limbs.  The  patient  may  utter  a  cry,  and  then  more 
or  less  suddenly  fall  or  sink  down  apparently  unconscious.  The 
fall  is  never  quite  sudden,  as  in  epilepsy,  nor  does  she  injure 
herself.  What  usually  happens  is  that  the  patient  throws 
herself  on  to  a  chair  or  couch,  and  then  slips  gradually  on 
to  the  floor.  Then  the  seizure  commences,  and  consists  of 
convulsive  movements — invariably  bilateral  —  affecting  the 
arms,  legs,  and  head.  The  patient  drums  on  the  floor  with  her 
heels,  throws  her  arms  about,  and  tosses  her  head  from  side  to 
side.  There  is  a  marked  tendency  towards  the  development  of 
opisthotonos.  The  eyes  are  deviated  upwards  and  inwards, 
in  some  cases  the  eyelids  are  firmly  closed ;  the  corneal 
reflex  may  be  abolished,  but  the  pupillary  light  reflex  is  not 
lost.  Tongue-biting  does  not  occur,  but  the  patient  may  bite 
her  lips  or  the  finger  of  an  onlooker.  The  character  of  the 
convulsions  varies  from  opisthotonic  rigidity  to  wild,  semi- 
purposive,  convulsive-like  movements.  There  is  no  deviation 
of  the  head  and  eyes  to  one  side,  as  in  epilepsy,  and  the 
rapid  alternation  between  the  opisthotonic  and  convulsive 
phases  is  quite  characteristic.  A  further  common  feature  is 
the  increase  of  the  movements  and  the  struggling  of  the 


526  NERVOUS  DISEASES 

patient,  when  an  effort  is  made  to  restrain  her.  If  a 
pin  be  placed  in  the  direction  in  which  the  movements 
are  taking  place,  a  diversion  of  the  action  not  uncommonly 
occurs. 

The  duration  of  the  seizure  varies  ;  it  is  rarely  less  than 
ten  minutes,  but  may  be  prolonged  from  a  half  to  three  or 
more  hours. 

Urine  is  rarely  passed  during  the  attack. 

Recovery  is  abrupt,  the  patient  suddenly  sits  up,  rubs  her 
eyes,  and  asks  where  she  is.  There  is  no  post-paroxysmal 
mental  confusion  or  automatism.  Memory  of  the  events  of 
the  fit  may  be  present,  but  more  commonly  there  is  only  a 
vague  idea,  or  no  recollection  of  it  at  all. 

3.  '  Hystero-epileptic'  seizures.  These  seizures  are  rare 
in  this  country,  but  are  not  infrequent  in  France,  and  it  is 
to  the  French  writers  that  we  owe  the  classical  descriptions 
of  '  la  grande  hysterie.'  In  many  of  the  attacks  loss  of 
consciousness  is  complete,  but  throughout  the  fit  it  may  be 
obvious  from  the  motor  phenomena  that  the  patient  is  acting 
a  subconscious  idea.  The  attack  may  commence  with 
opisthotonos,  the  body  resting  on  the  feet  and  occipital 
region  of  the  skull,  the  back  highly  arched,  and  the  belly 
prominent.  This  attidude  may  suddenly  give  place  to  one  of 
adoration,  in  which  the  patient  kneels  with  the  hands  clasped, 
and  the  head  and  eyes  thrown  upwards;  or  there  may  be 
the  assumption  of  a  crucifixion  position,  in  which  she  lies 
on  her  back  with  the  arms  abducted  and  outstretched  at 
right  angles  to  the  body.  The  attack  is  usually  followed 
by  delirium,  hallucinations,  cramps,  and  contractions  of  the 
limb.  The  whole  attack  may  last  from  a  quarter  up  to 
half  an  hour,  or  longer. 

4.  Automatic  phenomena.  These  are  phases  in  which 
the  patient  appears  to  be  in  a  dream,  and  may  carry  out  acts 
and  movements,  which  are  almost  impossible  under  normal 
conditions.  These  attacks  are  usually  started  by  a  severe 
emotional  shock.  They  are  sometimes  of  short  duration,  a 
few  hours,  or  may  be  prolonged  for  days  or  weeks.  They  are 
preceded  by  a  prodromal  stage  of  irritability  and  headache, 
and  may  be  succeeded  by  an  after-stage  of  exhaustion,  con- 
fusion, and  headache.  During  the  attacks  patients  may  travel 
long  distances,  and  awake  to  find  themselves  in  an  unknown 


HYSTERIA  527 

locality.  The  condition  more  closely  resembles  the  somnambu- 
listic state  than  any  other  ;  for,  during  the  phase,  complicated 
acts  and  movements  are  effected,  semi-purposive  in  intent, 
but  in  reality  automatic. 

A  condition  very  similar  to  the  above  is  found  in  epilepsy, 
as  well  as  in  alcoholism  and  states  of  mental  deficiency. 
According  to  Janet,  the  hysterical  character  of  the  attacks  is 
established  when  it  can  be  artificially  induced  and  a  recollec- 
tion of  it  revived  by  hypnotic  suggestion. 

5.  Trance  or  stupor.  This  condition  may  persist  for 
many  days,  the  patient  lying  quiet  and  often  presenting  a 
death-like  appearance.  The  skin  is  wax-like,  the  eyelids 
semi- closed,  the  respirations  superficial,  the  temperature 
slightly  subnormal,  and  the  heart's  action  feeble.  There  is, 
however,  often  a  suggestive  twitching  or  tremulousness  about 
the  eyelids,  and  the  pupils  respond  to  light.  In  the  slighter 
varieties  of  hysterical  sleep  the  patient  may  move  her  limbs, 
or  change  her  position,  or  even  mutter.  In  others,  the 
appearance  is  more  suggestive  of  the  cataleptic  state,  in  which 
the  limbs  are  in  a  condition  of  plastic  rigidity,  and  remain  in 
any  position  in  which  they  are  placed.  On  recovery,  there  is 
loss  of  memory  of  the  events  of  the  sleep  period. 

These  sleep  periods  are  caused  by  psychical  circumstances, 
and  may  be  either  of  sudden  or  gradual  onset ;  but  some  can 
be  induced  by  pressure  upon  the  hypnogenic  points —such  as 
the  breasts. 

Hypnotism  has  come  to  be  used  as  an  important  agent  in 
the  differential  diagnosis  of  the  automatic  states,  because  in 
hysterical  cases  automatism,  trance,  and  stupor  can  be  induced 
artificially  by  hypnotism ;  whereas  in  epilepsy,  psychasthenia, 
and  insanity,  hypnotism  fails  to  reproduce  the  symptoms. 

The  Cranial  Nerves 

Smell  and  taste.  These  may  be  diminished,  or  lost,  on 
one  side,  in  association  with  hysterical  hemianesthesia,  or 
abolished  on  both  sides. 

Vision.    The  following  are  the  visual  symptoms  of  hysteria  : 
(a)  Concentric    narrowing,    or    contraction    of    the   visual 
field.    This  may  be  unilateral  or  bilateral.    When  the  former,  it 
is  commonly  upon  the  same  side  as  the  cutaneous  anaesthesia, 


V's  NERVOUS  DISEASES 

the  field  being  reduced  to  a  circular  area  as  small  as  ten, 
or  even  five,  degrees  ;  if  bilateral,  it  is  more  marked  on  one 
side  than  the  other.  The  amblyopic  area  either  embraces  all 
forms  of  vision,  or  is  limited  to  colour  perception.  It  has  been 
stated  that  in  hysteria  the  area  for  red  remains  large,  and  is 
the  last  to  be  lost ;  while  in  colour  amblyopia  from  organic 
disease,  the  red  and  the  green  fields  are  impaired  early. 

(b)  Amaurosis,  or  hysterical  blindness,  is  more  often  uni- 
lateral and  upon  the  same  side  as  the  hemianesthesia.  It  is 
unaccompanied  by  any  change  in  the  optic  nerves.  It  is 
usually  of  traumatic  origin,  of  sudden  onset  and  disappear- 
ance, and  requires  great  care  in  the  diagnosis. 

Hemianopsia  and  central  scotoma  are  so  rare  in  hysteria 
(if,  indeed,  they  ever  occur),  that  the  existence  of  these  symp- 
toms usually  points  to  organic  disease. 

Photophobia  is  a  not  uncommon  hysterical  symptom. 

Hearing.  Hysterical  deafness  usually  incomplete  in 
character  -is  not  infrequent,  and,  if  unilateral,  is  found  upon 
the  side  of  the  motor  or  sensory  paralysis.  Being  a  cortical 
condition,  it  responds  to  the  tests  for  nerve  and  labyrinthine 
deafness— i.e.,  there  is  a  positive  Rhine  reaction,  and  the 
perosseous  and  aerial  conduction  are  synchronously  impaired. 
It  is  sometimes  combined  with  mutism.  There  may  be  an 
associated  anesthesia  of  the  external  auditory  meatus.  In 
organic  disease  of  the  labyrinth  there  is  tinnitus  aurium  and 
frequently  vertigo. 

Hysterical  hyperaceusis  is  an  affection  in  which  noises 
and  sounds  are  so  keenly  perceived  as  to  cause  mental  pain. 

Ocuh-motor  nerves.  Occasionally  there  is  an  apparent 
paralysis  of  the  conjugate  movements  of  the  globes.  In 
organic  paralysis  of  conjugate  movement,  a  patient,  who  is 
unable  voluntarily  to  move  the  eyes  to  one  or  other  side, 
can  only  follow  the  observer's  finger  by  turning  the  head 
in  the  direction  required.  In  hysteria,  on  the  other  hand, 
she  is  able  to  keep  her  eyes  fixed  upon  an  object,  when  the 
head  is  passively  moved  in  the  opposite  direction. 

An  appearance  of  false  ptosis  may  be  produced  by  spasm 
of  the  orbicularis  palpebrarum.  Paralysis  of  single  ocular 
muscles  probably  never  occurs  in  hysteria.  Nystagmus  is 
never  an  hysterical  symptom. 

Fifth  nerve.     Anaesthesia  over   the  face  and  head  may  be 


HYSTERIA 


529 


part  of  a  general  or  unilateral  affection  of  sensation.  If  it 
exists  apart  from  general  anaesthesia,  it  does  not  coincide  with 
the  sensory  distribution  of  the  nerve  on  the  skin,  but  reaches 
to  the  margin  of  the  jaw,  taking  in  that  portion  of  the  skin  of 
the  face,  which  is  supplied  by  the  cervical  nerves. 

Seventh   and    twelfth    nerves.     The   face   and   tongue   are 


^^m 


h 


Fig.  173. — Illustrates  hysterical  spasm  of  the  right  side  of  the  face. 


rarely  paralysed.  Both  may  be  affected  by  spasm.  Spasm 
of  the  tongue  on  one  side,  when  present,  is  a  characteristic 
hysterical  symptom. 

Hysterical  affections  of  speech.  These  may  occur  alone,  or 
in  association  with  other  hysterical  stigmata.  Hysterical 
mutism  is  rare,  and  is  closely  related  to  hysterical  aphonia. 
Both  may  be  induced  or  disappear  suddenly,  under  the 
influence  of  strong  emotional  excitement.  In  mutism,  the 
patient  is  unable  to  speak  at  all,  even  in  a  whisper ;  in 
aphonia,  whispering  is  preserved.  In  both  the  disability  is  in 
the  vocal  mechanism,  but  is  more  advanced  in  mutism  than 

34 


530 


NERVOUS  DISEASES 


in  aphonia  (Wyllie).  Stammering  may  also  be  found  in 
hysteria.  Sometimes  it  follows  an  attack  of  mutism  or 
aphonia.  It  may  be  accompanied  by  spasmodic  movements 
or  contortions  of  the  face,  jaw.  and  eyelids. 


Motoe  Phenomena 

The  motor  symptoms  of  hysteria  are  :  - 

1.  Paralyses  and  contractures. 

2.  Tremors. 

i.  Paralyses  and  contractures 

Paralyses  may  be  of  two  types ;  the  one  flaccid,  and  the 
other  associated  with  muscular  stiffness  and  contracture. 

The  flaccid  paralysis  is 
the  less  common  variety.  It 
is  characterised  by  a  limp  or 
flaccid  condition  of  the  para- 
lysed limb  or  limbs.  As  a 
rule  it  is  of  sudden  onset,  and 
almost  invariably  associated 
with  complete  loss  of  sensa- 
tion. The  muscles  are  not 
w7asted,  and  the  electrical 
reactions  are  normal.  The 
limb  may  be  passively  moved 
in  all  directions,  hypotonia  is 
not  present,  and,  if  tested  by 
the  state  of  the  tendon 
reflexes,  the  muscular  tone 
may  appear  to  be  increased. 
Voluntary  movement  is  com- 
pletely abolished,  and  when 
the  patient  is  asked  to  move 
the  limb,  no  effort  is  made 
to  do  so.  The  paralysis  is 
motion.  In  the  upper  limb, 
side  is  impossible  when  the 
arm  is  held  out  horizontally  from  the  shoulder,  yet  on 
coughing  the  latissimus  dorsi  is  seen  to  contract,  illustrating 
paralysis  of  voluntary  movement,  as   far  as  that  muscle  is 


Fig.  174. — A  case  of  hysterical  flaccid 
paralysis  affecting  the  right  arm. 

entirely    one    of    voluntary 
although    adduction     to    the 


HYSTERIA  53 1 

concerned.  If  a  patient  with  hysterical  hemiplegia  be 
asked  to  raise  herself  from  the  supine  position,  without 
using  her  arms,  the  paralysed  leg  may  remain  stationary 
on  the  hed,  while  the  non- paralysed  limh  rises.  This 
necessitates  the  active  contraction  of  the  muscles  of  the 
paralysed  limb  :  as,  normally,  on  attempting  to  sit  up,  the 
legs  are  actively  extended  at  the  hips,  in  order  to  provide  a 
fulcrum  for  the  flexor  action  of  the  abdominal  muscles.  If, 
in  organic  hemiplegia,  a  similar  attempt  is  made  to  sit  up, 


Fig.  175. — Hysterical  drop  -wrist ;  the  paralysis  is  of  the  flaccid  type. 

the  paralysed  leg  tends  to  rise  from  the  bed  to  a  greater 
extent  than  that  of  the  opposite  side. 

The  second  type  of  paralysis  is  associated  with  stiffness  of 
the  muscles.  This  is  the  more  common  form  of  hysterical 
palsy.  The  muscles  are  not  wasted,  and  may  be  slightly 
hypertonic.  The  voluntary  paralysis  is  less  complete  than 
in  the  flaccid  form,  slight  movement  being  usually  preserved. 
There  is  no  true  spasticity,  although  the  limb  may  be  held  in 
a  more  or  less  stiff  attitude  from  active  muscular  contraction. 
In  this  type  of  paralysis,  when  the  patient  is  asked  to 
perform  a  movement,  all  the  outward  evidence  of  great  effort 
is  demonstrated  in  associated  movements  of  the  face,  trunk, 
and  limbs. 

If,  for  example,  the  patient  is  asked  to  strongly  flex  the 

34  * 


532 


XKKVOUS  DISKASKS 


forearm,  and  the  observer,  supporting  the  forearm  in  one 
hand,  places  the  other  over  the  biceps  and  triceps  muscles, 
he  will  feel,  first,  a  well-marked  contraction  of  the  triceps, 
followed  by  a  feeble  contraction  of  the  flexors  of  the  forearm. 
If  the  arm  is  then  semi-flexed  at  the  elbow,  and  the  patient 
asked  to  extend  the  arm,  the  converse  will  be  observed  :  first 
a  tightening  of  the  biceps,  and  then  a  feeble  contraction  of 
the  triceps.  Similar  phenomena  may  be  seen  at  the  knee  or 
ankle  joints.  This  prior  contraction  of  the  antagonists  before 
the  prime  movers  is  a  highly  characteristic  phenomenon  of 
the  functional  palsies,  and,  as  Beevor  '  has  shown,  does  not 

occur  in  organic  affections. 
In  consequence  of  this, 
the  voluntaiy"movements 
present  a  jerky,  irregular 
action.  In  some  cases  the 
synergic  and  associated 
actions  of  the  paralysed 
limb  are  not  affected ;  in 
this  it  simulates  organic 
disease. 

In  distribution  the  para- 
lysis is  hemiplegic,  para- 
plegic, or  monoplegic.  In 
both  the  hemi-  and  para- 
plegic varieties  the  gait  is  usually  of  a  characteristic  type  ; 
and  the  paralysed  limbs  are  dragged  along  the  ground,  the 
feet  are  shuffled — no  attempt  being  made  to  raise  them  from 
off  the  floor ;  or  the  feet  are  placed  in  such  a  position  that 
the  dorsum  rests  upon  the  ground,  while  the  sole  points 
upwards  and  backwards. 

The  paralyses  are  often  of  long  duration,  lasting  months  or 
years,  but  are  as  a  rule  curable. 

Paralysis  is  of  the  cortical  type  in  that  movements  and 
not  muscles  are  affected. 

Contractures.  Certain  cases  of  hysterical  palsy  present 
well-marked  contractures.  These  are  due  to  active  contrac- 
tions of  various  muscles,  and  ma}^  simulate  the  positions 
observed  in  organic  spastic  contractures,  or  they  may  fix  the 


Fig.  170. — A  case  of  hysterical  paraplegia 
with  contractures  of  the  lower  limbs. 


1  Beevor,  Croonian  Lectures,  1903. 


HYSTERIA 


533 


limb  in  more  or  less  characteristic  hysterical  attitudes.  Con- 
tractures may  affect  the  whole  limb,  or  portions  only  of  a  limb. 
In  the  upper  extremity,  the  tendency  is  towards  flexion  of  the 
elbow,  wrist,  and  fingers,  with  adduction  of  the  arm  to  the 
side  of  the  body ;  in  the  lower  extremity,  towards  extension  at 
the  hip  and  knee  joints,  with  the  foot  in  a  position  of  talipes 
equino- varus.  In  such  cases  passive  movement  is  impossible, 
but    under   an   anaesthetic   the   contracture   passes   away,  a 


Fig.  177. — Illustrates  a  case  of  hys- 
terical contracture  of  the  hands 
and  fingers. 


Fig.  178. — A  case  of  hysterical 
contracture  of  the  right 
ankle  and  foot. 


phenomenon  which  does  not  occur  in  organic  contractures 
where  structural  alterations  have  taken  place.  Contractures 
are  not  confined  to  the  limbs.  One  half  of  the  tongue  may 
be  affected,  and  on  protrusion  is  deviated  to  the  same  side. 
An  appearance  of  ptosis  may  be  produced  by  contracture  of 
the  orbicularis  palpebrarum,  and  torticollis  may  result  from 
contracture  of  the  sterno-mastoid  muscle. 

A  form  of  motor  paralysis  occurring  in  hysteria  is  known 
as  astasia  abasia.  In  this  condition  there  is  no  loss  of  power 
of  movement  as  long  as  the  patient—  usually  a  young  person — 


534 


NERVOUS  DISEASES 


lies  in  bed,  but  as  soon  as  an  attempt  is  made  to  stand  or 
walk,  the  legs  give  way  and  a  fall  occurs.  Corresponding 
phenomena  are  seen  in  the  hands  and  arms.  Here  the 
patient  appears  to  have  full  power,  but  is  quite  unable 
to  use  a  needle,  play  the  piano,  or  hold  and  use  a  pen. 
It  has  been  shown  also  that  such  persons  may  move 
their  lips  in  the  ordinary  way,  but  are  unable  to  blow 
or  to  whistle. 


Fig.  179. — A  case  of  hysterical  contracture  of  the  right  foot. 


2.  Tremors 

Three  types  of  hysterical  tremor  are  found  : 

(a)  Vibratory  tremor.  This  is  a  fine  vibratory  tremor,  like 
that  seen  in  Graves's  disease,  and  alcoholism.  It  is  not 
under  the  control  of  the  will,  it  may  be  present  during  rest, 
and  is  increased  under  observation  and  when  the  hands  are 
outstretched. 

A  variety  of  the  above  is  a  coarse  vibratory  tremor.  It 
may  be  present  or  absent  during  rest,  and  is  increased  on 
voluntary  movement.  It  has  this  peculiarity— that  if  the 
tremor  is  arrested  by  holding  the  affected  limb  it  starts,  or  is 
increased,  in  another. 

(b)  Intention  tremor.  A  tremor  of  medium  rhythm, 
absent  during  rest,  but  occurring  on  voluntary  effort— similar 
to  that  seen  in  disseminated  sclerosis. 

(c)  Bhjjthmic  tremor.  A  slow  tremor  resembling  that  seen 
in  paralysis  agitans,  persisting  during  rest,  and  only  slightly, 
if  at  all,  under  voluntary  control.  A  more  or  less  persistent 
tremor,  which  is  confined  to  the  legs  and  has  the  features  of 
the  ankle  clonus,  may  also  be  observed. 


HYSTERIA  535 


Sknsoky  Phenomena 


Subjective  sensations  are  less  common  in  hysteria  than 
in  organic  nervous  disease.  When  present  they  are  chiefly  in 
the  form  of  pains.  The  common  seats  of  spontaneous  pain 
are  the  head,  back,  side  of  the  chest,  under  the  left  breast,  the 
epigastrium,  and  the  inguinal  regions.  Not  only  may  pain  be 
located  in  these  regions,  but  it  may  be  the  most  prominent 
symptom  in  association  with  hysterical  affections  of  the  joints, 
viscera,  breasts,  and  the  vertebral  column.  In  many  cases  the 
area  to  which  the  pain  is  referred  is  peculiarly  hyperaesthetic  ; 
so  that  light  pressure  causes  severe  pain,  while  deep  pressure, 
when  gradually  applied,  does  not  give  rise  to  discomfort.  In 
the  cases  with  paresthesia  or  numbness,  deadness,  and  cold- 
ness, neither  the  objective  sensory  loss  nor  the  motor  palsy  is 
complete. 

Tenderness  on  pressure  is  a  characteristic  feature  in 
hysteria,  and  is  to  be  distinguished  from  the  hyperesthesia  to 
contact  already  mentioned.  The  tender  areas  are  usually 
limited  in  extent ;  the  commonest  situations  are  the  left 
inguinal,  the  left  inframammary  and  epigastric  regions,  and 
various  points  along  the  spine,  and  sometimes  on  the  head. 

In  some  cases  pressure  exerted  upon  one  of  these  spots 
produces  an  hysterical  seizure,  in  consequence  of  which  the 
term  '  hystero-genetic  zones  '  has  been  applied  to  them. 

Objective  sensory  disturbance  is  by  no  means  universal 
in  hysteria,  but  is  one  of  its  most  constant  signs.  In 
contrast  to  the  objective  sensory  loss  of  organic  disease,. 
which  is  usually  recognised  by  the  patient  in  hysteria,  the 
patient  may  be  unaware  of  its  existence  ;  so  much  so,  indeed, 
that  the  opinion  has  been  expressed  that  the  loss  of  sensation 
may  have  been  suggested  by  the  examination.  This  is 
probable  in  some  cases,  and  under  no  circumstances  is  it 
warrantable  to  ask  patients,  if  they  '  feel  so  and  so,'  when 
each  test  is  applied,  but  rather  to  encourage  them  to  state 
what  the  sensation  conveyed  to  their  mind  is,  and  to  get  them 
to  locate  it. 

The  onset  of  anaesthesia  is  often  sudden,  being  induced  by 
an  emotional  shock,  or  injury,  or  following  upon  an  hysterical 
seizure.     It  is  a  mobile  form  of  anaesthesia,  shifting  from  one 


:,::<; 


NERVOUS  DISEASES 


to  another  part  of  the  body.  It  may  disappear  under  the 
influence  of  morphia,  alcohol,  and  chloroform  anaesthesia. 
It  may  persist  for  years,  and  sometimes  disappears  quite 
suddenly. 

The  following  method,  suggested  by  Janet,  is  useful  in 
demonstrating  hysterical  anaesthesia.  If  the  patient  is  told  to 
Bay  '  Yes,'  when  touched,  and  '  No,'  when  not  touched,  it  will 
frequently  happen  that  the  answer  '  No  '  is   given  as  often 


Fn;s.  ISO  ami  INI. —  Illustrate  the  front  and  back  views  of  a  case 
of  hysterical  anaesthesia.  Observe  that  the  sensory  loss  is 
incomplete  in  distribution,  and  that  in  front  it  passes  across 
the  middle  line. 


from  the   anaesthetic   side,  as  '  Yes '  from  the  aesthetic  side 
of  the  body. 

Another  test  is  the  following,  and  may  be  applied  when 
one  limb  is  paralysed.  In  many  hysterical  cases  the 
patient  is  obsessed  with  the  idea  of  paralysis,  and,  although 
when  tested  in  the  ordinary  way,  is  found  to  be  anaesthetic 
over  the  paralysed  arm,  yet  when  investigated  through  the 
motor  system,  the  hysterical  nature  of  the  condition  may  be 
fully  demonstrated.  She  is  first  asked  to  place  a  finger  of  the 
paralysed  hand  upon  any  place  where  she  is  touched.     She  is 


HYSTERIA 


53? 


then  touched  upon  some  part  of  the  aesthetic  side,  when  it 
may  happen  that  the  paralysed  arm  is  slightly  moved,  but, 
recognising  the  paralysis,  she  states  that  she  cannot  move  the 
arm.  The  examiner  at  once  tells  her  to  use  the  sound  arm 
instead,  and  proceeds  immediately  to  touch  her  on  some  part 
of  the  anaesthetic  area.  In  most  cases  the  patient,  without  any 
hesitation,  correctly  localises  the  spot  touched. 

It  should  be  pointed  out  that  these  apparent  inconsistencies 
are  not  due  to  malingering,  but  result 
from  the  definite  and  well-recognised 
psychical    condition,    which    is     the 
basis   of  hysteria. 

Loss  of  sensation  may  be  described, 
first  as  regards  its  character,  secondly 
its  degree,  and  thirdly  its  distri- 
bution. 

(a)  Character.  In  severe  cases, 
usually  associated  with  a  flaccid 
paralysis,  all  forms  of  sensation  may 
be  lost.  In  others,  the  sense  of  posi- 
tion and  muscle  pain  sensibility  may 
be  preserved,  with  loss  of  the 
cutaneous  sensibilities.  Most  com- 
monly all  forms  of  cutaneous  sensa- 
tion are  affected,  although  in  rare 
cases  a  dissociation  of  painful  and 
thermal  from  tactile  sensibility  has 
been  described. 

(b)  Degree.  The  loss  may  be  com- 
plete. In  the  majority  of  cases, 
however,  impairment  only  is  found. 
All  forms  may  be  recognised  correctly,  but  less  distinctly 
than  normal.  In  some  cases  the  impairment  of  cutaneous 
pain  sensibility  is  disproportionate  to  the  degree  of  impair- 
ment of  the  other  forms,  and  almost  amounts  to  analgesia. 
In  cases  with  incomplete  loss,  localisation  is  usually  fairly 
correct. 

(c)  Distribution.  Hysterical  anaesthesia  may  be  generalised 
over  the  whole  body.  It  may  extend  up  to  a  circular  limit 
round  the  neck.  It  may  be  unilateral,  involving  mucous 
membranes  and  the  special  senses  on  the  same  side.     It  may 


Pig.  182. — Chart  showing 
a  common  type  of  hys- 
terical henii-anaesthesia. 
The  dotted  areas  indicate 
where  the  sensory  loss 
was  incomplete. 


538  NERVOUS  DISEASES 

be  limited  to  an  upper  or  lower  limb,  or  to  both  upper  and 
both  lower  extremities.  When  limited  to  the  upper  limb  it 
may  involve  the  lingers  only,  or  extend  as  far  as  the  wrist, 
elbow,  or  shoulder,  or  to  the  middle  line  of  the  body.  The 
upper  limits  are  definite  and  usually  circular  in  the  transverse 
plane  of  the  limb  or  body.  In  some  cases  a  complete  loss 
may  extend  to  a  definite  line  round  the  wrist,  and  a  less 
complete  loss  to  a  definite  line  at  the  elbow.  These  are  the 
'glove'  anaesthesias.  Of  a  like  distribution  are  the  amesthesias 
of  the  lower  limb  ('  shoe,'  '  sock,'  or  '  stocking '  anaesthesia). 

It  is  obvious  that  these  areas  do  not  correspond  either  to 
nerves,  or  to  segmental  regions.  They  most  resemble  the 
anaesthesia  of  organic  cerebral  conditions  ;  but  are  distin- 
guished by  their  definite  upper  limit.  If  affecting  all  four 
limbs,  they  have  some  resemblance  to  the  anaesthesias  of 
peripheral  neuritis  and  early  stages  of  sub-acute  combined 
degeneration  of  the  cord,  in  which  conditions  the  upper  limit 
is  ill  defined  and  fades  into  normal  sensation.  Hysterical 
anaesthesia  may  also  be  patchy,  or  may  affect  opposite  quad- 
rants of  the  body,  such  as  the  arm  and  upper  part  of  the  body 
on  one  side,  and  the  leg  and  lower  part  of  the  body  on  the 
opposite  side.  Or  three-fourths  of  the  trunk  may  be  involved 
to  a  line  drawn  through  the  nipple.      (Fig.  180.) 

The  Reflexes 

The  tendon  reflexes  are  never  abolished;  as  a  rule  they 
are  increased.  A  true  ankle  clonus,  as  in  organic  disease, 
is  never  found.  A  spurious  ankle  clonus,  which  is  really  a 
form  of  rhythmic  tremor,  may  be  elicited,  but  when  present 
it  is  not  associated  with  spasticity.  It  may  commence  when 
the  muscular  tone  is  relaxed,  and  often  ends  suddenly. 

The  plantar  reflexes  are  never  extensor  even  after  an 
hysterical  seizure.  In  a  large  number  of  cases  the  reflexes 
are  absent,  usually  in  association  with  anaesthesia.  In  many 
cases  they  are  present,  and  although  sometimes  diminished, 
are  flexor  in  type. 

The  abdominal  reflexes  are  present,  but  in  cases  with 
anaesthesia  over  the  abdomen  they  may  not  be  elicited. 

The  corneal  reflex  may  be  diminished,  but  if  tested  by 
blowing  upon  the  eye  of  the  anaesthetic  side,  both  eyes  blink. 


HYSTERIA  539 

The  pharyngeal  irritability  is  diminished  or  lost. 

Retention  of  urine  is  a  not  infrequent  symptom  of  hysteria, 
but  incontinence  of  a  persistent  type  is  unknown.  Similarly, 
although  constipation  is  common,  incontinence  of  feces  never 
occurs. 

Local  Manifestations  of  Hysteeia  simulating  Organic 

Disease 

Anorexia  nervosa  may  be  induced  in  some  hysterical  girls 
by  a  hypersensitiveness  to  criticism  and  ridicule  as  to  their 
getting  stout.  In  other  cases  it  arises  from  no  obvious  cause. 
It  consists  of  a  persistent  refusal  of  food,  and  a  gradual  loss 
of  appetite,  impaired  digestion,  occasionally  associated  with 
vomiting,  and  finally  a  state  of  profound  inanition.  The 
patient  becomes  a  '  living  skeleton,'  with  extreme  reduction  of 
bodily  and  mental  power.  Its  onset  is  most  common  in  girls 
during  the  period  of  puberty,  and  ma.y  continue  for  several 
years,  or  even  throughout  life. 

Hysterical  vomiting  is  more  frequent  than  anorexia,  and 
may  eventually  lead  to  a  severe  form  of  inanition,  although  it 
may  persist  for  a  long  time  without  giving  rise  to  marked 
impairment  of  nutrition.  Its  commencement  is  usually  de- 
pendent upon  an  organic  condition — such  as  gastric  ulcer, 
gastritis,  or  flatulent  distension  of  the  stomach.  The  vomit- 
ing occurs  immediately  after  food,  and  is  unassociated  with 
effort,  pain,  or  nausea.  It  eventually  becomes  a  species  of 
tic,  in  so  far  as  it  may  persist  after  the  original  exciting 
cause  has  been  removed ;  and  if  it  is  prevented  or  arrested, 
a  state  of  mental  anguish,  or  even  a  hysterical  seizure  is 
induced. 

Hysterical  flatulence  is  a  very  common  phenomenon.  It 
arises  in  two  ways  :  either  as  a  result  of  globus,  or  of  a  tic  of 
swallowing  ensuing  upon  the  globus  hystericus.  By  the 
repetition  of  this  act  the  stomach  is  filled  with  saliva  and  air, 
and  rapidly  becomes  distended.  Eructations  follow,  produc- 
ing in  the  patient  a  pleasurable  sensation.  The  nature  of 
this  affection  may  be  demonstrated  by  asking  the  patient, 
when  the  stomach  has  been  emptied,  to  continue  to  eructate. 
She  at  once  then  commences  to  swallow  air,  and  then  to  bring 
it  up  again.      In  other  cases    '  wind-sucking,'   analogous   to 


540  NERVOUS  DISEASES 

what  is  seen  in  horses,  may  he  ohserved.  The  air  is  sucked 
into  the  stomach,  and  the  patient  may  rapidly  distend  hoth 
the  stomach  and  the  intestines.  This  may  occasion  a  pro- 
nounced flatulent  distension,  amounting  to  a  phantom  tumour. 
In  one  such  case  voluntary  distension  of  either  the  stomach 
or  the  intestines  was  possible,  and  the  patient  was  able  to 
pass  the  ingested  air  from  the  intestines  back  into  the  stomach. 
Perforation  has  been  known  to  occur  in  such  cases. 

Dysphagia  is  due  to  spasm  of  the  oesophagus,  and  is  a 
rarer  condition  than  the  hysterical  vomiting  or  flatulence. 

Respiratory  symptoms.  Cough,  barking,  intermittent 
respiration,  hiccough,  yawning  are  all  of  the  nature  of 
hysterical  tics.  Polypnoea,  or  phenomenal  frequency  of  the 
respirations,  has  been  known  to  follow  severe  emotional 
shock. 

'  Hysterical  spine.'  A  frequent  manifestation  of  hysteria 
is  the  condition  known  as  '  hysterical  spine.'  It  is 
characterised  by  subjective  pain,  which  may  be  referred 
to  the  whole  length  of  the  back,  or  to  certain  more 
localised  portions  of  it.  It  is  common  over  the  lower 
sacral  and  coccygeal  regions  (coccygodynia),  over  the 
lower  and  mid-dorsal  regions,  and  the  cervical  region.  The 
pain  may  be  of  a  more  or  less  continuous  aching  character, 
which  is  generally  relieved  by  rest  upon  the  back.  On  exam- 
ination of  the  spine  no  curvature  is  present,  but  in  many 
cases,  owing  to  general  weakness,  a  postural  lateral  distortion 
of  the  spinal  column  may  be  observed.  Angular  curvature 
never  arises  from  hysteria.  An  apparent  loss  of  mobility  may 
be  seen.  The  inorganic  (functional)  character  of  this  distor- 
tion may  be  demonstrated  by  asking  the  patient  to  stand 
upright  with  the  feet  together,  and  then  to  bend  forward  with 
both  arms  equally  extended  as  if  to  touch  the  feet,  when  the 
postural  curvature  immediately  disappears.  Tests  by  aid  of 
lateral  rotation  movements  may  also  be  applied. 

On  percussion  of  the  spine,  tenderness  may  be  general  or 
localised.  It  may  be  more  pronounced  on  pressure  over  the 
muscles  on  one  or  other  side  of  the  vertebral  spines.  It  is 
also  characteristic  that  the  tenderness  is  as  acute  on  light  as 
on  deep  pressure. 

The  two  conditions  which  simulate  hysterical  spine  are 
tuberculous  caries  and  malignant  disease  of  the  vertebras.    The 


1IYSTKK1A 


541 


former  may  be  recognised  by  the  local  position  of  the  pain  and 
by  the  immobility  of  the  spine,  by  the  localised  tenderness  to 
deep  pressure  and  on  movement,  by  a  local  prominence  of  one 
or  more  vertebrae,  and  by  evidence  of  pressure  upon  the 
nerve  roots  or  spinal  cord.  An  evening  rise  of  temperature 
would  be  in  favour  of  tuberculous  disease.  In  doubtful  cases 
an  X-ray  photograph  ought  to  be  taken. 

More  treacherous  is  the  simulation  of  hysteria  by  malignant 
disease  of  the  spine 
Such  cases  usually 
occur  after  forty  years 
of  age,  and  are  often 
associated  with  some 
degree  of  cachexia  and 
loss  of  weight  without 
obvious  cause.  In  the 
early  stages  pain  may 
be  the  only  symptom, 
and  may  be  referred 
to  parts  of  the  body 
other  than  the  spine. 
The  history  of  previous 
operative  interference  for 
the  removal  of  a  growth 
in  any  part  of  the  body 
— especially  the  breast, 
the  orbit,  and  the  pelvic 
organs — should  warn  the 
observer  against  the 
hasty  diagnosis  of  func- 
tional disorder.  Lumps 
or  growths  on  any  of  the 
other  bones  —  especially 
the  ribs  —  visceral  tu- 
mours, or  enlarged  glands,  should  in  all  cases  be  especially 
sought  for. 

Hysterical  joints.  The  commonest  form  is  seen  in  simu- 
lation of  hip-joint  disease,  which  may  arise  suddenly  from  some 
emotional  cause  or  trauma.  It  is  characterised  by  pain  inten- 
sified by  any  form  of  movment,  tenderness  on  palpation  with 
protective  contraction  of  the  surrounding  muscles.     Although 


ustrates  a  case  of  hysterical 
contracture  of  the  left  hip,  simulating 
disease  of  the  hip  joint. 


542  NERVOUS  DISEASES 

apparenl  shortening  of  the  limb  occurs  in  these  cases,  from  the 
position  in  which  the  limb  is  maintained,  no  real  shortening 
is  found  on  measurement.  Tenderness  may  be  associated 
with  hyperesthesia  over  wide  areas  around  the  joint.  The 
X-ray  examination  reveals  no  evidence  of  organic  joint 
affection,  and  under  anesthesia  the  joint  is  normally 
movable.  In  these  cases  there  is  an  absence  of  constitutional 
symptoms,  and  other  manifestations  of  hysteria  are  usually 
present. 

A  word  of  warning  may  be  said  about  the  diagnosis  of 
'hysterical  knee,' as  pain  in  the  knee  without  organic  affection 
of  that  joint,  is  frequently  the  first  and  only  sign  of  hip-joint 
disease. 

1  Hysterical  breast '  is  not  common  ;  pain  is  usually 
confined  to  one  organ,  and  no  sign  of  organic  disease  is 
apparent. 

Cardio-vascular  system.  Palpitation,  and  increased  action 
of  the  heart  are  common,  and  may  be  associated  with  globus 
as  the  aura  of  an  hysterical  seizure.  Cardiac  pain,  sometimes 
associated  with  faintness,  may  simulate  angina  pectoris,  but 
the  absence  of  vascular  degeneration  and  the  exciting  cause  of 
the  attack,  are  usually  sufficient  grounds  on  which  to  form 
a  diagnosis. 

Vaso-motor  and  trophic  symptoms.  The  most  prominent 
of  the  vasomotor  phenomena  is  an  abnormal  or  unusual 
pulsation  of  the  arteries.  This  is  especially  observed  in  the 
abdominal  aorta,  and  may  persist  without  change  for  months 
or  years. 

A  curious  phenomenon,  recognised  for  many  years  as  a 
feature  of  hysteria,  is  that  no  bleeding  occurs  when  the  skin 
of  the  paralysed  limbs  is  pricked  or  incised. 

In  hysteria  the  paralysed  limbs  are  frequently  blue  and 
cold,  or  of  a  mottled  appearance.  A  form  of  cedema  which  does 
not  pit  on  pressure,  and  which  may  be  most  marked  in  the 
morning,  has  also  been  described. 

Two  entirely  opposite  phenomena  in  the  secretion  of  urine 
are  also  observed  in  hysteria.  One  is  the  copious  flow  of  pale 
limpid  urine  which  follows  an  hysterical  seizure,  and  the  other 
the  suppression  of  the  urinary  secretion,  which  has  been  known 
to  persist  for  a  number  of  consecutive  days. 


HYSTERIA 


543 


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544  NERVOUS   DMvYSttS 

Diagnosis 

Hysteria  has  to  be  diagnosed  from  a  number  of  maladies 
of  an  organic  nature.  The  diseases  with  which  it  may  be 
confounded,  and  the  main  points  in  differential  diagnosis,  are 
detailed  in  the  Table  on  p.  543. 

Prognosis  and  course 

The  phenomena  of  hysteria  are  essentially  curable.  They 
have  been  known  to  persist  for  many  years,and  yet  under 
suitable  treatment  to  have  completely  and  permanently 
disappeared. 

The  disease  shows  a  remarkable  tendency  to  remission  and 
relapse  of  symptoms  -a  feature,  however,  by  no  means  peculiar 
to  it,  as  it  is  also  an  outstanding  characteristic  of  disseminated 
sclerosis,  a  malady  with  the  early  stages  of  which  hysteria  is 
most  apt  to  be  confused. 

In  the  common  hysterical  symptoms  such  as  fits  and 
paralysis — occurring  in  young  persons  from  emotional  shock  — 
the  outlook  as  regards  recovery  is  most  satisfactory  ;  also  in 
traumatic  cases,  when  the  question  of  compensation  has  been 
settled.  It  is  notorious,  on  the  other  hand,  that  traumatic 
hysteria  may  persist  for  an  indefinite  time  pending  the 
settlement  of  a  claim. 

It  is  rare  for  hysteria,  or  any  of  its  manifestations,  to 
cause  death,  but  this  has  been  known  to  occur  in  severe  cases 
of  anorexia  from  exhaustion  and  inanition. 

Treatment 

In  dealing  with  the  hysterical  person  of  either  sex, 
certain  principles  ought  to  be  always  kept  in  mind  by  the 
physician.  In  the  first  place,  it  is  important  for  successful 
treatment  that  he  should  obtain  both  the  confidence  and  the 
co-operation  of  his  patient.  He  should  neither  make  light 
of  symptoms  which  appear  important  to  the  sufferer,  nor 
magnify  others  which  are  of  only  slight  import ;  and,  above 
all,  he  should  enter  into  the  details  of  the  treatment  with 
care,  thoroughness,  and  knowledge. 

In  the  second  place,  as  the  carrying  out  of  the  treatment, 


HYSTERIA  545 

sometimes  a  long  and  tedious  process,  devolves  upon  nurses 
or  attendants,  the  physician  should  satisfy  himself  that  these 
are  persons  sufficiently  competent  and  trustworthy  to  execute 
his  orders,  and  are  at  the  same  time  sufficiently  tactful  to 
manage  those  whose  emotional  state,  for  the  time  being  at 
all  events,  requires  to  be  handled  with  skill,  patience,  and 
authority. 

There  is  only  one  method  universally  applicable  for  the 
treatment  of  hysteria  which  is  likely  to  lead  to  a  successful 
result,  viz.  isolation.  This  is  the  basis  of  the  well-known 
method  of  Weir  Mitchell,  in  which  the  patient  is  removed  from 
all  intercourse,  personally  or  by  correspondence,  with  relatives 
and  friends.  For  the  proper  carrying  out  of  this  system,  it 
is  essential  that  the  patient  be  removed  to  a  nursing  home, 
institution,  or  hospital,  and  placed  under  the  care  and  super- 
vision of  a  tactful  and  thoroughly  responsible  nurse  or  atten- 
dant. Such  complete  isolation  is  essential  in  the  majority  of 
cases  of  hysteria,  whatever  may  be  the  cause  or  the  particular 
symptoms  of  individual  cases.  Various  modifications  are 
occasionally  employed,  such  as  sending  the  patient  away  from 
home  under  care  of  a  nurse,  or  isolating  her  with  a  nurse 
in  one  of  the  rooms  of  the  house,  but  these  are  rarely  so 
satisfactory. 

Having  effected  the  isolation  of  the  patient,  it  is  neces- 
sary to  apply  certain  therapeutic  methods  and  agents.  In 
the  first  place  it  is  important  that  the  patient  should  be 
treated  by  complete  rest  in  bed  over  a  period  of  four  to  six 
weeks  or  longer. 

With  a  view  to  the  improvement  of  general  nutrition,  it 
is  important  that  abundant  nourishment  be  given,  more 
especially  in  the  form  of  milk — three  to  eight  pints  daily. 
Massage  may  be  applied,  either  once  daily  or  more  often, 
over  a  period  of  six  weeks,  two  months,  or  more,  according  to 
the  necessities  of  particular  patients. 

Medicinal  remedies  alone  are  of  little  value  in  the  treat- 
ment of  hysteria,  but  may  be  of  use  for  the  relief  of  symptoms, 
or  as  temporary  expedients.  Of  these  the  most  efficacious 
are  the  preparations  of  valerian,  assafcetida,  the  bromides 
in  small  doses,  iron,  and  arsenic.  Tonic  remedies,  such  as 
cod-liver  oil,  Easton's  syrup,  and  quinine,  may  be  given  from 
time  to  time  or  during  convalescence. 

35 


546  NERVOUS  DISEASES 

In  hysterical  paralysis  and  contracture,  the  application  of 
the  faradic  current  to  the  lirnhs  and  hack  is  often  of  valuahle 
assistance  in  assisting  to  restore  motion  and  sensation. 
During  hysterical  convulsions  the  use  of  a  strong  faradic 
current  conveyed  through  a  wire  brush  and  applied  generally 
may  modify  or  cut  short  a  seizure. 

In  conjunction  with  the  ahove  methods  spinal  douches,  or 
the  alternate  application  of  hot  and  cold  water  to  the  hack, 
for  a  few  minutes  each  morning  are  often  of  service. 

It  has  already  been  stated  that  hysterical  patients  are 
susceptible  to  the  influence  of  suggestion.  Acting  upon 
this  element  in  the  mental  state  of  hysteria,  many  physicians 
have  regarded  hypnotic  suggestion  as  a  satisfactory  therapeutic 
remedy.  It  has  been  urged,  however,  that  the  employment  of 
this  agent  is  likely  to  intensify  a  feature  of  the  disease  which 
it  is  intended  to  cure.  Our  own  experience  of  the  remedy 
has  been  limited,  but  those  who  have  made  a  study  of  the 
subject  speak  favourably,  not  only  of  the  immediate  effects, 
but  of  the  lasting  results  which  are  induced. 

AKINESIA  ALGERA 

This  term  is  given  to  an  affection  in  which  the  patient  is 
unable  to  move  on  account  of  pain,  or  from  the  onset  of  pain 
on  movement,  for  which  no  appreciable  cause  can  be  found. 
Three  varieties  are  described :  (a)  complete  akinesia  algera  ; 
(b)  dyskinesia  algera,  in  which  gentle  movements  are  still 
possible  ;  and  (c)  apraxia  algera,  in  which  speaking,  reading, 
wTriting  or  thinking  are  impossible,  owing  to  the  development 
of  pains  in  the  head.  It  may  persist  for  months  or  years, 
and  in  one  case  which  we  have  studied  the  patient  was  bed- 
ridden for  seven  years,  but  ultimately  recovered  completely. 

The  clinical  condition  is  quite  definite,  although  the 
nature  of  the  malady  is  obscure.  Mobius,  who  first  described 
it,  regarded  the  pains  as  being  of  hysterical  nature,  but  they 
are  probably  of  a  paranoic  or  psychical  character. 


Part  XVI 

NEURASTHENIA 

Neurasthenia  is  a  condition  generally  associated  with  a 
lowered  state  of  the  general  health  and  characterised  by 
symptoms  of  mental  and  physical  fatigue,  usually  accompanied 
by  subjective  sensory  symptoms,  but  without  any  evidence  of 
organic  disease  of  the  nervous  system. 

Etiology.  Neurasthenia  is  a  disease  of  adult  life,  being 
most  frequently  met  with  between  the  ages  of  twenty-five  and 
fifty.  Both  sexes  are  affected,  but  the  disease  is  more  common 
among  men  than  women. 

Heredity.  Heredity  undoubtedly  plays  a  part  in  the 
causation  of  the  disease,  and  although  it  is  unusual  to  obtain 
a  history  of  grave  nervous  disorders,  such  as  epilepsy  or 
insanity  in  the  parents,  yet,  in  the  majority  of  cases,  there  is 
a  history  of  '  nervousness '  or  '  headaches '  in  one  or  other 
parent.  Perhaps  more  important  is  the  inheritance  of  a 
weak  constitution,  the  patient  being  liable  to  various  ailments 
and  easily  '  run  down.'  Alcoholism  and  consanguinity  in  the 
parents  is  noted  in  a  small  proportion  of  the  cases. 

Predisposing  causes.  Anything  which  tends  to  lower  the 
general  vitality  is  liable  to  bring  on  the  disease.  Thus  it  is 
that  in  most  cases  some  definite  physical  derangement  is 
present,  either  of  a  temporary  or  permanent  nature,  such 
as  dyspepsia,  constipation,  anseruia,  vaso-motor  disturbance, 
or  phosphaturia.  It  ought  not  to  be  forgotten  that  symp- 
toms of  neurasthenia  may  be  early  signs  of  grave  organic 
disease,  such  as  dementia  paralytica,  melancholia,  malignant 
disease,  tuberculosis,  Addison's  disease,  myasthenia  gravis,  or 
renal  disease.  Of  the  acute  diseases,  influenza  and  typhoid 
fever  are  specially  liable  to  be  followed  by  neurasthenia. 

In  young  women  the  strenuous  combination  of  study  with 

547  35  * 


548  NERVOUS  DISEASES 

social  indulgences  frequently  tends  to  the  production  of  the 
malady.  Excessive  child-bearing,  prolonged  lactation,  and 
the  climacteric  period  favour  its  development  in  some  women, 
but,  on  the  other  hand,  the  menopause  in  many  cases  rings 
down  the  curtain  upon  neurasthenia  of  many  years'  standing. 

Immediate  causes.  The  most  common  is  overwork  attended 
with  worry,  and  this  perhaps  accounts  in  part  for  the  greater 
prevalence  of  the  disease  in  urban  than  in  rural  districts. 
Prolonged  stress  and  strain  are  other  fruitful  sources  of 
neurasthenia,  as  was  seen  in  many  of  those  who  served  in  the 
late  South  African  war,  and  as  is  commonly  noted  in  those 
who  break  down  after  nursing  some  friend  or  relative  during 
a  long  illness. 

Excesses  of  various  kinds,  more  particularly  of  the  sexual 
functions,  emotional  shocks  and  physical  injuries  may  all 
contribute  to  the  development  of  neurasthenia. 

Traumatic  neurasthenia  is  a  term  applied  loosely  to  cases 
in  which  after  injuries,  sometimes  of  a  trivial  character, 
symptoms  of  neurasthenia  or  functional  paralysis  develop. 
These  symptoms  are  due  to  the  fright  or  shock  attendant 
upon  the  injury  rather  than  to  any  lesion  of  the  nervous 
tissues  or  their  coverings.  If  these  cases  are  examined  closely 
they  may  be  divided  into  two  classes :  (1)  those  in  which 
the  symptoms  are  neurasthenic,  and  (2)  those  in  which  the 
symptoms  are  hysterical  or  functional.  Traumatic  neuras- 
thenia is  of  special  importance  from  the  medico-legal 
aspect,  as  it  not  infrequently  forms  the  basis  of  claims  for 
compensation. 

Symptoms.  Neurasthenia  is  characterised  by  a  number 
of  symptoms,  psychical  and  physical,  mainly  of  a  subjective 
character.  They  are  all,  however,  dependent  upon  an  exhausted, 
debilitated,  or  irritable  nervous  system. 

The  preponderance  of  the  physical  or  psychical  symptoms 
depends  largely  upon  the  social  and  mental  status  of  the 
patient.  In  brain  workers  mental  symptoms  are  most 
obtrusive,  but  in  labourers  and  those  whose  work  is 
physical  rather  than  mental,  bodily  symptoms  are  the  more 
troublesome. 

Psychical  symptoms.  The  patient  gradually  becomes 
conscious  that  his  brain  fatigues  more  easily,  that  he  is 
unable  to  concentrate  his  attention  upon  his  work,  that  his 


NEURASTHENIA  549 

memory  has  become  impaired,  and  that  he  tires  readily  over 
simple  exercises,  which  formerly  gave  satisfaction  and  enjoy- 
ment. In  this  state  he  usually  resorts  to  '  pick-me-ups,'  or 
seeks  relief  in  physical  or  social  distractions,  but  without 
success.  Neither  work  nor  pleasure  interests  him,  he  is 
irritable  over  trifles,  easily  worried,  and  cannot  make  a  decision 
on  even  the  most  trivial  matters.  He  starts  to  work  full  of 
energy  and  good  intentions,  which  however  dissolve  whenever 
he  is  confronted  with  his  task.  He  attempts  to  do  something 
else,  and  again  finds  that  his  energy  and  will  power  fail  him. 
He  becomes  depressed,  and  various  fears  and  apprehensions 
begin  to  oppress  him.  He  cannot  sleep  properly  ;  if  he  falls 
asleep  on  going  to  bed,  he  wakes  up  in  the  early  hours  of  the 
morning,  or  if  after  lying  awake  all  night  he  gets  some  sleep 
in  the  early  morning,  he  wakes  up  unrefreshed  and  miserable. 
As  a  general  rule  he  feels  better  and  brighter  in  the  evening, 
but  dreads  the  approach  of  night  time.  In  this  condition 
he  falls  an  easy  prey  to  quack  literature.  He  reads  about  his 
symptoms,  and  his  mind  becomes  filled  with  and  obsessed 
by  apprehensive  imaginings.  His  conversation  is  entirely 
devoted  to  detailing  his  symptoms,  and  he  seeks  to  get  sym- 
pathy from  all  he  comes  in  touch  with.  He  cannot  take 
food,  and  eventually  becomes  a  misery  alike  to  himself  and 
his  friends. 

The  examination  of  the  nervous  system  reveals  the  charac- 
teristic mental  state — loss  of  brain  power  and  mental  energy, 
nervousness,  depression,  and  irritability.  In  other  cases 
there  is  a  placid,  self-satisfied  air  of  resignation,  but  in  both 
types  there  is  an  overwhelming  desire  on  the  part  of  the 
patient  to  describe  in  the  most  minute  detail  every  symptom, 
ache  and  pain,  requiring  on  the  part  of  the  physician  great 
forbearance  and  patience,  lest  he  give  the  idea  that  he  thinks 
there  is  nothing  wrong. 

The  cranial  nerves  are  unaffected. 

The  motor  system  shows  general  loss  of  power  and  prone- 
ness  to  fatigue.  There  may  be  varying  degrees  of  fine  or 
coarse  vibratory  tremor,  but  there  is  neither  spasticity  nor 
flaccidity.  Observation  usually  intensifies  the  symptoms. 
The  electrical  reactions  are  normal. 

Sensory  system.  Subjective  sensations  are  referred  chiefly 
to  the  head,  back  and  legs,  and  are    of   every   imaginable 


550  NERVOUS  DISEASES 

character.  The  most  common  have  been  already  mentioned. 
Objective  sensory  changes  are  much  less  common,  and  usually 
consist  of  a  diminution  rather  than  a  loss  of  sensation. 
The  chief  exceptions  to  this  rule  are  seen  in  cases  of 
traumatic  paralysis,  which  are  described  under  hysteria 
(p.  580). 

The  reflexes.  The  deep  reflexes  are  almost  invariably  in- 
creased, and  a  knee  jerk  may  sometimes  be  obtained  by  tapping 
the  shin.  In  these  cases  the  eliciting  of  the  jerk  may  cause 
the  patient  a  'jar  at  the  spine '  and  cause  him  to  start.  With 
the  increase  in  the  deep  reflexes,  however,  there  is  no  corre- 
sponding spasticity,  and  clonus  is  not  present.  The  superficial 
reflexes  are  usually  exaggerated.  The  plantar  reflexes  are 
liexor  in  type.     The  sphincters  are  not  affected. 

Clinical  types.  Some  symptoms  of  neurasthenia  form  the 
outstanding  feature  of  particular  cases,  so  that  certain  types 
have  been  described. 

1.  Cases  in  which  headache  of  a  persistent  character  is  the 
outstanding  s37mptom. 

2.  Cases  in  which  gastric  symptoms  preponderate,  dyspepsia 
of  a  specially  troublesome  and  protracted  form,  or  increase  in 
the  secretion  of  hydrochloric  acid,  or  falling  down  of  the 
stomach  (gastroptosis). 

3.  Cases  in  which  the  circulatory  system  is  especially 
prone  to  disturbances  of  a  functional  character.  In  these, 
palpitation,  rapid  action  of  the  heart,  tremors  of  the  hands, 
suggesting  Graves's  disease,  and  breathlessness  on  exertion 
are  observed. 

4.  Another  type  is  seen  especially  in  young  men,  in  which 
the  symptoms  are  referred  to  the  generative  organs  and  the 
sexual  functions.  In  these  cases  nocturnal  emissions  are 
frequent  and  are  often  accompanied  by  a  prostatic  discharge. 
This  type  is  associated  with  marked  depression,  hypochon- 
driacal feelings  and  imaginings  of  sexual  impotence.  Sexual 
desire  may  be  diminished  and  the  sexual  act  imperfect  from 
feeble  erection  and  premature  emission.  The  persistence  of 
these  symptoms  eventually  leads  to  distress  of  mind,  fatigue 
and  exhaustion,  want  of  decision  and  distaste  of  work,  and 
not  infrequently  considerable  insomnia. 

The  physical  condition  in  neurasthenia  is  sometimes  not 
materially  affected.     There  are  some  cases  in  which  loss  of 


Neurasthenia  551 

body  weight  is  well  marked.  Other  patients  are  anaemic 
and  badly  nourished.  Constipation  is  frequent,  and  in  most 
cases  some  flatulence  and  dyspepsia  exist  apart  from  definite 
dilatation  and  gastroptosis.  The  extremities  are  sometimes 
blue  and  cold. 

The  heart's  action  is  frequently  accelerated,  but  there  is 
rarely  any  irregularity,  and  the  pulse  is  regular  and  of  fair 
volume. 

The  temperature  is,  as  a  rule,  subnormal. 

The  urine  is  usually  small  in  quantity,  phosphates  are 
frequently  present  in  considerable  quantities,  and  abnormal 
quantities  of  oxalate  of  lime,  uric  a.cid,  and  of  indican  have 
been  noted. 

Differential  diagnosis  of  neurasthenia  from  disorders 
presenting-  neurasthenic-like  symptoms  The  diseases  from 
which  neurasthenia  has  to  be  distinguished  are  :— 

1.  The  psychoses  of  puberty  and  adolescence — dementia 
precox. 

2.  Mild  or  modified  types  of  mania  and  melancholia — ■ 
(manic-depressive  insanity) . 

3.  The  early  (neurasthenic)  stage  of  some  types  of  paralytic 
dementia. 

4.  Psychasthenia. 

5.  Organic  diseases  of  the  nervous  or  other  systems. 

1.  The  psychoses  of  puberty  and  adolescence.  Neuras- 
thenia in  its  true  form  is  unknown  during  puberty  and  is 
rarely  seen  in  the  early  years  of  adolescence.  Symptoms  of  a 
neurasthenic-like  type,  therefore,  arising  during  these  epochs 
should  be  viewed  with  suspicion  and  the  prognosis  guardedly 
given. 

The  symptoms  of  these  psychoses  consist  of  excitement 
or  depression,  occasionally  attended  by  delusions  or  fancies 
of  a  fleeting  nature.  Suicidal  tendencies  are  not  uncommon. 
After  the  continuance  of  such  symptoms  for  an  indefinite 
time,  spontaneous  recovery  or  varying  degrees  of  dementia 
may  supervene.  In  other  cases  the  symptoms  are  more 
obviously  hysterical,  or  of  a  psychasthenic  character  accom- 
panied by  insomnia.  Their  prominent  characteristic  is  a 
tendency  towards  relapse  and  remission. 

2.  Mild  or  modified  types  of  mania-melancholia.  This 
type  of  psychosis  is  common  during  adult   life,  and  in   its 


552  NERVOUS  DISEASES 

milder  tonus  may  readily  be  mistaken  for,  and  is  frequently 
treated  as,  neurasthenia.  Although  this  psychosis  presents 
the  exhaustion,  feebleness  of  will  power,  and  want  of  energy 
so  well  shown  in  neurasthenia,  the  chief  feature  by  which 
it  may  be  distinguished  from  the  latter  is  the  marked 
tendency  towards  melancholy  or  mental  depression.  It  is 
often  accompanied  by  considerable  insomnia.  The  majority 
of  patients  suffering  from  this  type  of  psychosis  will  give  a 
history  of  one  or  more  previous  attacks. 

It  is  rare  in  young  people,  but  not  uncommon  throughout 
adult  life  and  at  the  climacteric  epoch  in  women. 

3.  The neurasthenic  type  of  early  paralytic  dementia.  This 
occurs  chiefly  in  adult  males  who  have  had  syphilis.  Such 
cases  may,  or  may  not,  present  physical  signs  of  organic 
disease,  such  as  loss  of  the  tendon  jerks,  or  reflex  pupillary 
immobility.  If  these  signs  are  found  in  association  with 
neurasthenic  sj^mptoms,  the  onset  of  paralytic  dementia  may 
almost  with  certainty  be  diagnosed.  The  failure  to  obtain 
these  signs  of  organic  disease  does  not  necessarily  exclude  the 
onset  of  paralysis ;  therefore  in  those  cases  a  lumbar  puncture 
ought  to  be  done  and  a  cytological  examination  made  of  the 
cerebro-spinal  fluid.  If,  on  examination  of  the  centrifuged 
deposit,  the  lymphocyte  count  shows  150  to  200  or  more 
lymphocytes,  the  diagnosis  of  general  paralysis  may  be 
made  with  complete  assurance.  Although  an  increase  of  the 
lymphocytes  is  present  in  most  cases  of  cerebro-spinal  syphilis, 
yet  the  count  is  rarely  so  high  as  in  the  parasyphilitic  diseases. 

4.  Psychasthenia.  This  type  of  psycho-neurosis  as  found 
in  young  adults  is  largely  of  a  remitting  character,  while  in 
the  later  years  of  life  it  may  be  more  persistent  and  chronic. 
It  is  primarily  characterised  by  the  obtrusiveness  of  certain 
morbid  fancies  and  fears,  apprehensions,  fixed  ideas  and 
obsessions.  It  is  less  likely  to  be  confounded  with  neurasthenia 
than  those  conditions  just  described. 

5.  Organic  diseases  of  the  nervous  or  other  systems.  In 
every  case  presenting  neurasthenic  symptoms  a  most  complete 
examination  is  necessary,  and  this  should  be  repeated  at 
intervals. 

Course  and  prognosis.  Neurasthenia  runs  a  prolonged 
course,  sometimes  lasting  many  years.  Its  duration,  how- 
ever, depends  to  a  large  extent  upon  the  cause.     If  due  to  a 


MYASTHENIA  5&3 

temporary  and  removable  stress  or  strain,  recovery  under 
suitable  treatment  may  occur  within  a  few  months.  But  the 
temperament  which  favours  the  development  of  the  disease 
usually  favours  the  prolongation  of  the  symptoms.  If  the 
patient  is  in  a  position  to  abandon  work,  to  undergo  a  thorough 
'  rest  cure,'  or  to  take  a  sufficiently  extended  change,  recovery 
usually  results. 

In  cases  attributed  to  toxic  causes,  such  as  influenza,  enteric 
fever,  or  other  post-febrile  states,  the  prognosis  is  good ;  in 
those  associated  with  pyorrhoea  and  rheumatoid  arthritis, 
the  prognosis  is  that  of  the  causal  condition. 

In  traumatic  cases  the  '  rest  cure  '  finds  one  of  its  most 
useful  applications.  Should  an  action  for  compensation  be 
impending,  there  is  little  chance  of  recovery  until  this  is 
settled ;  when  settled  the  recovery  is  in  most  cases  rapid  and 
complete. 

Cases  of  '  sexual  neurasthenia '  are  persistent  and  of  an 
inveterate  character,  owing  to  the  tendency  towards  hypochon- 
driasis and  mental  depression. 

Treatment.  The  treatment  of  neurasthenia  is  based 
upon  the  efficient  carrying  out  of  rest,  both  mental  and 
physical.  For  this  purpose  the  '  rest  cure,'  as  described  by 
Weir  Mitchell,  is  of  great  use.  The  rest  cure  consists  of 
isolation,  rest  in  bed,  massage  and  abundance  of  nourishment, 
preferably  in  the  form  of  milk.  It  is  usually  carried  out  in 
homes  situated  preferably  in  the  country,  so  as  to  permit  of 
the  patient  being  carried  out  into  the  open  air.  All  cases  of 
neurasthenia,  however,  do  not  respond  satisfactorily  to  this 
method.  Men  are  less  amenable  to  the  treatment  than  women, 
and  in  them  a  modified  course  may  be  found  sufficiently 
efficacious.  In  the  modified  rest  cure,  isolation  is  not  essen- 
tial, while  rest  may  be  judiciously  combined  with  mild  forms 
of  outdoor  recreation.  Cases  of  neurasthenia  complicated 
with  pronounced  insomnia,  or  with  much  mental  depression, 
are  not  favourable  for  the  rest  cure. 

An  important  means  of  treatment,  particularly  in  the 
early  stages  of  the  disease,  or  in  mild  cases,  consists  in  send- 
ing the  patient  away  from  work  and  enjoining  a  change  of 
scene.  All  forms  of  climate  are  not,  however,  suitable  to 
neurasthenics,  and  the  choice  of  a  satisfactory  locality  is  often 
a  matter  of   difficulty.     Just  as  in  health  some  individuals 


554  NERVOUS  DISEASES 

favour  the  seaside  more  than  the  higher  altitudes,  so  in 
neurasthenia  the  selection  of  the  locality  should  be  deter- 
mined by  what  the  patient  has  found  most  beneficial  when  in 
health.  A  point  of  importance  in  this  matter  is  the  avoidance 
of  long  railway  journeys,  and  of  frequent  changes  from  one 
place  to  another.  For  this  reason  many  neurasthenics  who 
are  sent  abroad,  or  upon  a  voyage  round  the  world,  return 
not  only  with  no  material  benefit,  but  often  with  a  decided 
aggravation  of  their  symptoms. 

All  forms  of  outdoor  recreation,  if  not  carried  to  excess  and 
to  the  production  of  plrysical  fatigue,  are  to  be  encouraged. 
To  the  worker  in  the  town,  two  or  three  afternoons  a  week 
given  to  golf,  or  riding  exercise,  may  keep  off  the  onset  of 
neurasthenic  symptoms. 

Drugs  in  the  treatment  of  neurasthenia  are  of  little  use, 
except  to  relieve  temporary  symptoms.  In  young  women 
perhaps  most  advantage  is  to  be  obtained  from  the  glycero- 
phosphates of  lime  and  soda,  in  other  cases  Easton's  syrup 
acts  as  a  powerful  and  satisfactory  tonic.  Of  others  which 
ma}7  be  mentioned,  mix  vomica,  quinine,  the  mineral  acids, 
the  hypophosphites,  valerian,  iron  and  arsenic  are  of  most 
service.  In  cases  presenting  much  restlessness,  with  palpita- 
tion and  a  tendency  towards  insomnia,  the  bromides  find  one 
of  their  most  useful  applications.  They  ought  not,  however, 
to  be  used  for  more  than  a  short  time.  In  the  type  of  case 
characterised  by  headache,  analgesics  such  as  antipyrin, 
phenacetin,  and  aspirin  may  be  advantageously  given  from 
time  to  time. 

In  cases  presenting  symptoms  of  either  gastroptosis  or 
dilatation,  a  carefully  regulated  diet  with  abdominal  massage 
will  afford  relief,  attention  being  paid  to  the  mouth  and  teeth. 
In  the  event  of  hyperchlorhydria  being  present,  the  excess  of 
acid  may  be  neutralised  by  hot  water,  or  by  a  natural  alkaline 
water  such  as  that  of  Yichy. 

Hydrotherapy  and  electricity  may  both  be  useful  in  other- 
wise intractable  cases  of  neurasthenia.  Neither  of  them  should 
be  relied  upon  solely,  but  may  be  prescribed  as  auxiliary 
methods. 

The  only  certain  and  satisfactory  treatment  of  genuine 
neurasthenia  is  complete  mental  and  physical  rest,  combined 
with  attention  to  the  complicating  features  already  described. 


Part  XVII 

PSYCHASTHENIA 

This  is  a  psycho-neurosis  characterised  by  the  presence  of 
a  variety  of  symptoms  of  which  nervous  fears,  apprehensions, 
and  obsessions  are  the  most  prominent.  It  is  often  accom- 
panied by  periodic  seizures  of  a  vertiginous  character,  which 
are  combined  with  head  sensations,  aches,  and  pains.  Another 
feature  of  the  malady  is  a  peculiar  dazed  reverie  or  dream 
state,  in  which  objects  and  persons  do  not  seem  to  be  real, 
but  which  is  not  associated  with  any  loss  of  consciousness, 
such  as  occurs  in  epilepsy. 

Etiology.  The  disease  is  commonest  between  the  ages  of 
twenty-one  and  forty,  but  may  be  found  both  during  puberty 
and  after  fifty.  It  is  about  equally  distributed  between  the 
sexes,  and  seems  to  stand  in  some  way  to  the  development  of 
the  sexual  functions,  being  found  in  its  most  typical  forms 
during  adolescence.  It  is  as  frequent  amongst  hospital  as 
private  patients. 

A  hereditary  neuropathic  history  is  not  always  obtainable, 
but  when  present  will  be  found  to  be  chiefly  in  the  direction 
of  parental  alcoholism  and  '  nervousness.' 

The  psychopathic  nature  of  this  malady  is  seen  in  the 
nervous  symptoms  presented  by  the  relatives  of  many  of 
these  patients,  such  as  epilepsy,  insanity,  and  alcoholism, 
and  in  such  nervous  associations  as  periodic  headaches, 
hysterical  manifestations,  somnambulism,  occasionally  chorea, 
tic-like  movements,  nervous  dyspepsia,  and  the  traumatic 
neuroses. 

Symptoms.  These  are  in  part  temperamental  or  congeni- 
tal and  in  part  acquired.  The  former  constitute  the  psych- 
asthenic or  ideo-obsessive  constitution,  which  is  common 
to  most  psycho-neurotic  persons ;  the  latter  are  seen  in  the 


556  NERVOUS  DISEASES 

development  of  morbid  fears,  obsessions,  and  states  of  reverie 
or  dreaminess. 

Of  the  obsessions,  an  enormous  assortment  is  described  by 
patients.  Only  a  few  common  instances  need  be  given. 
Sometimes  they  are  merely  general  fears  and  apprehensions, 
as  of  '  something  going  to  happen  '  ;  fears  of  being  alone,  of 
being  in  the  dark,  of  being  in  a  crowd,  or  in  a  confined  space, 
or  they  may  have  reference  to  some  special  phenomenon  such 
as  becoming  insane,  or  paralysed,  or  of  dying.  A  curious 
apprehension  is  found  in  a  fear  of  falling  through  the  bed, 
or  the  floor,  or  of  sinking  through  the  ground.  Or  again, 
sufferers  from  this  affliction  describe  a  feeling  of  apprehensive- 
ness  that  they  may  inflict  injuries  upon  themselves  or  others, 
when  they  handle  knives  or  razors.  Sometimes  these  sen- 
sations become  so  engrossing  that  a  fear  of  homicide  or  of 
suicide  is  present,  although  self-destruction  rarely  occurs. 

Allied  to  the  above  are  states  of  'panic,'  such  as  sudden 
desires  amounting  to  impulsive  actions  to  get  out  of  a  train  in 
motion,  or  to  escape  from  a  crowded  church  or  theatre. 

In  other  instances  the  obsession  takes  the  form  of  a 
paralysis  of  action,  the  patient  being  afraid  to  go  about,  but 
yet  dreading  being  alone ;  or  an  utter  inability  to  decide 
upon  a  particular  course  of  action. 

Others  are  tormented  by  conscientious  scruples,  or  by 
ideas  that  a  word  lightly  spoken  may  have  led  to  harmful 
consequences. 

Probably  of  the  same  fundamental  nature  as  the  above, 
and  of  greater  significance,  are  the  drug  habits- — dipsomania, 
morphinomania,  and  cocainomania.  Various  moral  delin- 
quencies may  also  be  grouped  under  this  heading,  amongst 
which  the  sexual  perversions  may  be  mentioned. 

The  reverie  or  dream  states  of  psychasthenia  have  to  be 
distinguished  from  somewhat  similar  conditions  occurring 
as  the  aura  of  epileptic  seizures.  In  psychasthenia  they 
consist  of  spells  of  dazedness,  in  which  the  patient  feels 
as  if  he  is  in  a  dream.  During  this  phase  there  is  a  feeling 
of  unreality,  sometimes  referred  to  objects  and  people  around 
him ;  at  other  times  to  his  own  organs  and  body.  These 
patients  state  that  they  have  no  real  existence,  that  they  do 
not  seem  to  have  any  body,  or  that  their  organs  have  gone. 
Others  suffer  the  feeling  that  '  they  are  not  what  they  are,' 


PSYCHASTHENIA  557 

that  nothing  makes  any  impression  upon  them ;  they  even 
doubt  whether  what  they  see  and  hear  are  real. 

The  psychasthenic  condition  is  also  accompanied  in  many 
instances  by  periodic  attacks,  which  seem  to  occupy  a  position 
somewhere  on  the  '  borderland  '  of  epilepsy.  Although  these 
may  occur  during  sleep  and  wake  the  patient,  they  are  never 
associated  with  loss  of  consciousness  or  convulsion.  To  some 
of  these  seizures  the  term  '  psycholepsy '  has  been  applied  by 
Janet.  They  consist  of  attacks  of  giddiness,  throbbing,  and 
palpitation  of  the  heart,  a  feeling  of  choking,  a  sense  of  fear 
and  sometimes  of  impending  death.  They  may  be  accom- 
panied by  excessive  perspiration  and  followed  by  great 
exhaustion.  Their  duration  may  be  from  a  few  to  several 
minutes.  In  our  opinion  these  attacks  are  not  primarily 
psychical,  but  are  the  consequences  of  functional  disturbances 
of  the  lower  centres  in  the  medulla  oblongata. 

The  head  sensations  and  other  paresthesia  are  numerous 
and  varied,  such  as  giddiness,  feelings  of  a  burning  character, 
sensations  of  emptiness  in  the  head,  or  as  if  the  head  was 
opening  and  shutting,  or  of  tightness  round  the  head.  Some- 
times there  are  feelings  as  of  something  trickling  down  the 
back  of  the  head,  or  moving  about  within  the  brain,  or 
of  confusion  in  the  head.  Others  describe  a  sensation  of 
bursting,  or  'popping,'  inside  the  head. 

These  sensations  may  or  may  not  be  accompanied  by  mental 
depression. 

When  the  symptoms  are  intense,  or  at  the  commencement 
of  a  seizure,  a  feeling  of  sinking  in  the  epigastrium  is  often 
present,  and  gives  rise  to  the  impression  that  the  disease 
originates  in  this  locality.  Dyspepsia,  however,  is  not 
common,  although  flatulence  and  constipation  are  occasional 
accompaniments. 

Pain  in  the  head  is  rare,  so  also  is  insomnia,  although 
the  nights  may  be  disturbed  by  bad  dreams  and  some 
tendency  to  restlessness. 

Although  fatigue  is  readily  induced  on  exertion,  and  an 
inability  to  concentrate  the  attention  for  any  length  of  time 
may  be  present,  neither  of  these  symptoms  occupies  the  out- 
standing position  which  is  characteristic  of  neurasthenia. 
The  memory  may  be  impaired  temporarily  during  the  persis- 
tence of  the  symptoms.     One  of  the  features  characteristic  of 


558  NERVOUS  DISEASES 

the  malady  is  the  inability  to  stand  the  effects  of  alcohol. 
Although  the  symptoms  may  be  for  a  time  relieved  by  it, 
most  cases  present  a  marked  idiosyncrasy  towards  its  con- 
tinued use. 

In  the  psychasthenic  condition  there  is  an  absence  of 
physical  signs  of  nervous  disorder.  The  reflexes  are  un- 
altered, although  the  knee  jerks  may  be  slightly  exaggerated. 
There  is  no  tenderness  on  pressure  over  the  head  or  along 
the  spine.  Occasional  subjective  circulatory  symptoms  such 
as  palpitation,  blushing,  and  morbid  flushings  are  observed, 
and  a  proclivity  towards  flatulence,  discomfort  after  food,  and 
constipation  have  been  already  noted.  The  tongue  is  usually 
clean,  but  has  been  found  to  be  furred  and  coated  in  some 
cases  in  which  the  onset  of  the  symptoms  is  sudden  and  their 
progress  acute. 

Prognosis.  The  course  and  prognosis  of  the  malady  is 
variable.  When  it  occurs  in  adolescents  and  young  adults 
—where  it  is  seen  in  its  most  characteristic  forms — it 
presents  remissions  and  relapses  as  its  main  peculiarities. 
Under  favourable  conditions  it  is  a  curable  malady,  but 
running  a  prolonged  course  over  many  years.  When  it 
arises  'de  novo'  in  middle  life,  the  prognosis  is  less  good, 
as  it  is  more  apt  to  become  confirmed,  and  to  be  subservient 
to  the  varying  conditions  of  overwork,  anxiety,  shock,  worry, 
or  ill-health. 

There  is  no  tendency  towards  dementia.  Temporary 
depression  may  occasionally  supervene,  more  especially  at 
certain  epochs,  such  as  pubert}7,  adolescence,  and  the  climac- 
teric in  women,  but  the  underlying  obsessive  phenomena  are 
not  altered.  The  persistence  of  fears  and  apprehensions  may 
give  rise  to  a  condition  of  hypochondriasis  and  depression. 

Treatment.  The  treatment  of  psychasthenia  and  the 
ideo-obsessive  state  is  fraught  with  numerous  difficulties. 
In  the  first  place,  the  symptoms  do  not  lend  themselves 
to  amelioration  by  means  of  drugs,  although  valerian  and  its 
preparations  are  more  likely  to  be  of  assistance  than  any 
other  remedies,  especially  if  combined  with  small  doses  of 
the  bromides. 

The  rest  cure,  which  is  so  valuable  a  therapeutic  agent  in 
the  treatment  of  neurasthenia,  is  more  likely  to  aggravate 
the   symptoms   in  the  psychasthenic.     There  are,  however, 


PSYCH  ASTHENIA  559 

cases  in  which  nervous  and  physical  fatigue  form  prominent 
symptoms,  and  in  which  a  modified  rest  cure  may  bo  of 
distinct  service. 

For  the  treatment  of  this  condition,  congenial  surround- 
ings are  very  important.  These  patients  should  give  up 
work  for  a  time  and  lead  an  open-air  life  with  suitable 
exercise  and  amusement.  It  is  important  to  advise  a  com- 
plete change,  as  the  malady  is  not  infrequently  induced  by 
uncongenial  and  sedentary  employment. 

Psychotherapy,  or  treatment  by  means  of  suggestion,  has 
been  employed  advantageously  in  a  number  of  cases.  In 
many  the  results  are  encouraging,  and  the  method  should  be 
used  in  those  persons  in  whom  other  methods  of  treatment 
have  been  of  little  or  no  avail. 


Part  XVIII 

EPILEPSY 

Epilepsy  is  a  chronic  progressive  disease,  characterised  by 
the  periodic  occurrence  of  seizures,  in  which  loss  of  conscious- 
ness is  an  essential  feature,  commonly,  but  not  invariably, 
associated  with  convulsion,  and  frequently  accompanied  by 
psychical  phenomena. 

It  generally  occurs  in  persons  with  a  hereditary  neuro- 
pathic history,  which  may  show  itself  in  signs,  or  stigmata, 
of  degeneration  ;  it  runs  its  course  uninterruptedly,  or  with 
remissions,  over  a  number  of  years,  and  it  terminates  either 
in  a  cure,  in  the  establishment  of  the  confirmed  disease,  in 
delusional  insanity,  or  in  dementia. 

Etiology 

Epilepsy  is  a  prevalent  disorder,  and  although  its  frequency 
varies,  according  to  the  obtainable  statistics  from  different 
countries,  it  may  be  stated  that,  on  an  average,  two  persons 
in  every  thousand  of  the  population  are  epileptic.  Males  are 
more  commonly  afflicted  than  females,  the  greater  frequency 
of  the  male  sex  not  necessarily  depending  upon  the  greater 
stress  and  strain  to  which  this  sex  is  exposed,  but  being  a 
feature  of  the  disease  from  infancy  to  old  age. 

Heredity.  The  great  predisposing  cause  of  epilepsy  is  an 
inherited  neuropathic  predisposition.  A  family  history,  more 
especially  of  epilepsy,  alcoholism  and  insanity,  is  obtainable 
in  about  50  per  cent,  of  the  cases. 

The  hereditary  transmission  of  epilepsy  may  be  direct  from 
parent  to  offspring,  or  the  disease  may  reveal  itself  in  col- 
lateral relatives,  such  as  uncles,  aunts,  and  cousins.  Two  or 
more   members  of  the  same   family   may   be  afflicted,    and 

560 


EPILEPSY  561 

periodic  headache,  '  bilious  '  attacks,  migraine,  and  chorea  may 
be  found  in  other  members. 

Age  at  onset.  The  commencement  of  epilepsy  is  dated 
from  the  occurrence  of  the  first  fit— a  fact  which  is  sometimes 
difficult  to  ascertain,  as  it  may  occur  during  sleep,  or  assume 
a  form  which  is  not  recognised  as  of  epileptic  character.  The 
greatest  number  of  cases  of  epilepsy,  commencing  in  any 
single  year,  is  found  during  the  first  twelve  months  of  life. 
There  is  then  a  rapid  and  extensive  fall  in  the  number  of 
cases  to  the  fourth  year,  from  which  there  is  a  slight  increase 
up  to  the  seventh  year.  A  small  decline  occurs  about  the 
eighth  or  ninth  year,  preliminary  to  a  steady  increase  in  the 
disease,  which  reaches  a  maximum  between  the  ages  of 
twelve  and  fifteen.  From  this  period  onwards,  there  is  a 
decrease  in  numbers,  with  a  slight  temporary  increase 
between  the  twenty-fifth  and  twenty-eighth  years. 

Although  there  is  no  age  which  may  be  regarded  as  exempt 
from  epilepsy,  the  onset  of  fits  is  more  common  during 
certain  epochs,  which  may  thus  be  defined  :  (a)  from  birth  to 
eight  or  nine  years— corresponding  to  the  periods  of  rapid 
brain  growth  and  development  ;  (b)  from  the  tenth  to  the 
twenty-third  year — the  period  of  development  and  maturation 
of  the  reproductive  organs.  The  explanation  of  the  onset  of 
epileptic  fits  is  to  be  found  in  the  normal  instability  of 
the  nervous  system  during  these  two  periods,  acting  upon  a 
constitution  predisposed  by  heredity  to  convulsions. 

In  women  the  onset  of  epilepsy  is  almost  always 
accompanied  by  irregularity  in  the  catamenia,  and  the 
relation  between  '  the  period '  and  the  fit  incidence  is 
well  known.  In  close  association  with  this  function  is  the 
influence  which  pregnancy,  the  puerperium,  and  lactation  have 
upon  the  onset  or  course  of  epilepsy.  These  incidents  in  the 
life  of  a  woman  may  be  a  cause  of  epileptic  seizures,  or  they 
may  induce  a  relapse  after  a  long  remission  from  fits  ;  on  the 
other  hand  pregnancy  and  child-bearing  have  been  known  to 
favourably  influence  the  course  of  epilepsy. 
The  causes  of  epilepsy  are  : — 

1.  A  neuropathic  heredity  as  already  mentioned. 

2.  The  normal  instability  of  the  nervous  system  during 
certain  epochs  in  those  predisposed  by  heredity. 

3.  Miscellaneous    exciting    causes    acting    in    conjunction 

36 


562  NERVOUS  DISEASES 

with  a  neuropathic  heredity  and  the  normal  instability  of  the 
developmental  epochs.  Amongst  such  causes  are  :  (1)  Refl,ex 
influences  — arising  from  disease  of  the  nose,  eyes,  ears, 
teeth,  stomach,  and  intestines  (including  the  influence  of 
intestinarworms),  and  from  the  genital  organs.  (2)  Psychical 
causes— such  as  fright,  mental  emotion,  prolonged  anxiety, 
grief,  and  overwork.  (3)  Infective,  pyrexial,  and  toxic 
causes.  The  most  important  of  these  are  scarlet  fever, 
alcohol,  and  tobacco.  (4)  Seizures  of  an  epileptic  character 
may  be  associated  with  organic  disease  of  the  brain.  This 
does  not  constitute  epilepsy  as  here  defined. 

The  following  are  the  organic  conditions  in  which  gene- 
ralised epileptic  fits  may  occur :  (a)  defective  development 
of  the  nervous  system  in  infancy  and  childhood  (p.  212)  ; 
{b)  cerebral  lesions  in  infancy  and  childhood  (p.  216)  ; 
c  syphilitic  affections  of  the  brain  (p.  370)  ;  (d)  vascular 
diseases  of  the  brain  (p.  186)  ;  (e)  tumours  (p.  226)  ;  (J) 
hydrocephalus  and  general  increase  of  intracranial  pressure 
(p.  288)  ;   (g)  injury  to  the  head. 

Pathology 

The  macroscopical  appearances  of  the  brains  of  epileptics 
vary.  The  brain  is  usually  large,  the  convolutionary  develop- 
ment simple,  and  the  membranes  not  adherent  to  the 
cortex.  A  foam-like  or  frothy  exudate  may  be  seen  lying 
between  the  membranes  and  the  surface  of  the  cortex.  More 
or  less  sclerosis  of  the  cortex  is  present,  especially  of  the 
outer  layers  and  of  the  subcortical  white  matter  in  cases  of 
old-standing  epilepsy  complicated  with  dementia.  This 
sclerosis  is  best  seen  in  the  region  of  the  cornu  ammonis, 
which  is  atrophied  in  considerably  more  than  half  the  cases 
of  this  disease.  Atrophy  of  the  optic  thalamus,  more  com- 
monly upon  the  left  side,  has  been  described.  Other  portions 
of  the  brain  — such  as  the  cerebellum  and  the  medulla 
oblongata— may  also  be  atrophied.  Small  petechial  hemor- 
rhages, a.ngeiomata  or  blood  tumours,  some  thickening  of  the 
arterial  walls,  and  engorgement  of  the  blood-vessels  have 
also  been  occasionally  noted. 

The  microscopical  appearances  vary  to  a  large  extent, 
but  the  following  changes  have  been  observed :— 


EPILEPSY  563 

(a)  Changes  which  are  accom/pa/niments,  or  stigmata  of  a 
defectively  developed  nervous  system. 

Diminution  in  the  number  and  size  of  the  nerve  cells  of 
the  outer  layers  of  the  cerebral  cortex  has  been  described 
The  nucleus  of  the  large  pyramidal  cells  is  displaced  and  the 
cell  body  presents  a  granular  appearance.  The  persistence  of 
nerve  cells  in  the  white  matter  and  outer  layers  of  the  cortex 
—a  feature  common  to  the  lower  vertebrates,  new-born  infants, 
and  imbeciles— has  also  been  found. 

(b)  Changes  which  are  probably  the  direct  consequences  of  the 
seizures. 

Increase  of  the  neuroglia,  especially  of  the  first  cortical 
layer  and  the  subcortical  white  matter,  has  been  observed 
in  old-standing  cases  of  epilepsy.  A  patchy  sclerosis  is  also 
found  in  other  parts— such  as  the  optic  thalamus,  occipital 
lobes,  cerebellum,  medulla  oblongata,  and  cornu  ammonis.  It 
is  associated  with  a  great  increase  in  the  neuroglia  cells. 

The  blood-vessels.  The  veins  are  engorged  and  the  arteries 
collapsed  and  tortuous.  The  lymph  spaces  are  widely  dilated. 
Hyaline  spheres  or  masses  and  fibrin  threads  are  seen  lying 
freely  in  the  lumen  of  the  blood-vessels,  not  only  of  the  cortex, 
but  also  in  the  cerebellum,  medulla  oblongata,  and  spinal  cord. 

Chemical  pathology.  Urine.  This  presents  normal 
features,  except  that  after  a  severe  seizure,  or  a  series 
of  fits,  albumen  may  be  temporarily  present.  The  uric  acid 
excretion  is  stated  to  be  considerably  lessened  before  a  fit 
and  increased  afterwards  (Haig,  Krainsky).  Observers  are 
at  variance  as  to  whether  the  toxicity  of  the  urine  in 
epileptics  is  greater  than  in  normal  persons. 

Blood.  The  general  characters  are  those  of  an  average 
type  of  chlorosis.  A  slight  leucocytosis  is  constant,  and  is 
markedly  increased  after  a  fit.  A  sudden  fall  in  alkalinity 
occurs  prior  to  and  after  a  fit  (Pugh).  There  would  appear  to 
be  an  increased  tendency  to  coagulation  of  the  blood. 

Cerebrospinal  fluid.  Cholm  has  been  found  in  this  fluid 
in  epileptics,  probably  as  a  result  of  the  fits.  Lymphocytosis 
has  been  occasionally  observed. 

Thymus  gland.  This  gland  has  been  found  enlarged  in  a 
number  of  cases. 

The  examination  of  the  fluids  and  secretions  of  the  body 
do  not  afford  an  entirely  satisfactory  basis  on  which  to  build 

36  * 


564  NERVOUS  DISEASES 

a  theory  of  auto-intoxication.  Evidence  of  a  toxic  condition 
of  the  blood  exists  in  hyper-leucocytosis  and  in  a  tendency  to 
intravascular  clotting,  conditions  which  are  present  in  some 
of  the  clinical  types  of  epilepsy.  Other  views  of  the  auto- 
toxic  causation  of  epileptic  fits  are  Haig's  uric  acid  theory, 
Krainsky's  carbamate  of  ammonium,  Donath's  cholin,  and 
Ceni's  cytotoxins.  Of  none  of  these  is  there  certain  proof:  the 
evidence,  such  as  it  is,  pointing  to  these  substances  as  being 
the  effects  rather  than  the  cause  of  epileptic  seizures. 

Symptomatology 

The  symptoms  may  best  be  described  under  the  two 
subheadings  into  which  epilepsy  may  be  clinically  divided  : 
the  convulsive  and  the  psychical  elements. 


The  Convulsive  Element 

This  constitutes  the  fit  in  its  numerous  and  varied  mani- 
festations. In  order  to  understand  and  appreciate  the 
manifold  features  of  the  epileptic  seizure,  the  doctrine 
propounded  by  Herpin  may  be  stated,  that  the  incomplete 
attacks — cramps,  spasms,  giddinesses,  or  partial  convulsions — 
which  occur  irregularly  in  the  intervals  between  the  major 
attacks,  are  the  complete  seizures  reduced  to  their  initial 
symptoms,  and,  however  diversified  they  may  be,  are  always 
or  nearly  always  similar  in  the  same  subject. 

For  descriptive  purposes  the  fit  may  be  subdivided  into : 

1.  The  aura. 

2.  The  incomplete  fit. 

3.  The  complete  fit. 

The  aura,  or  warning,  is  the  initial  symptom  of  the  attack, 
or  may  constitute  the  whole  seizure. 

The  incomplete  attack,  or  minor  fit,  consists  of  the  aura, 
with  a  further  development  towards  the  phenomena  of  the 
complete  seizure — a  type  of  attack  which  may  or  may  not  be 
associated  with   loss  or  impairment  of  consciousness. 

The  complete  attack,  or  major  lit,  forms  the  classical 
epileptic  seizure,  in  which,  with  or  without  warning,  the 
victim  falls  from  sudden  loss  of  consciousness  and  is  convulsed. 

The  warning  spreads  almost  always,  though  not  invariably, 


EPILEPSY  505 

from  the  periphery  of  the  body  towards  the  head.  The 
sensation  ascends  from  the  hands  or  feet,  from  the  thoracic, 
epigastric  or  abdominal  organs,  or  from  the  organs  of  special 
sense.  In  many  cases  it  is  noted  that  when  the  sensation 
reaches  the  head  consciousness  is  abolished.  Sometimes, 
however,  consciousness  is  abolished  at  an  early  stage  of  the 
fit  :  at  other  times  general  convulsion  commences  before 
consciousness  is  lost,  but  in  the  majority  the  loss  of  con- 
sciousness occurs  synchronously  with  the  onset  of  muscular 
spasm  or  convulsion. 

i.  Auras  and  aura  fits 

Any  part  of  the  body  may  theoretically  be  the  starting- 
point  of  a  seizure,  but  there  are  certain  localities  in  which 
the  onset  is  more  common.  The  warning  may  commence  in 
one  or  other  of  the  following  ways,  or  the  entire  seizure  may 
be  confined  to  the  warning  sensation. 

(a)  Sensations  referred  to  the  stomach,  abdomen,  or  chest. 
These  consist  of  various  abnormal  sensations  —  such  as 
cramps,  spasms,  and  sometimes  pain,  occasionally  feelings  of 
suffocation  or  of  cardiac  distress. 

(b)  Head  sensations— such  as  pains,  'jumping'  or  'horrid 
feelings  '  in  the  head  ;  or  of  something  '  striking  the  brain,'  &c. 

(c)  Sensations  of  a  psychical  character  — such  as  dreads, 
sense  of  fear,  &c— which  are  occasionally  associated  with  a 
dreamy  state  or  a  feeling  of  unreality  or  of  non-existence. 

(d)  Subjective  or  perverted  sensations  of  special  sensibility, 
olfactory,  gustatory,  auditory,  and  visual  warnings.  It  is 
especially  in  association  with  the  olfactory  sensations  that  the 
dreamy  state  is  present— a  symptom  which  has  been  found 
to  arise  from  disease  of  the  uncinate  gyrus  (p.  255). 

(e)  Sensations  referred  to  the  limbs  and  body— such  as  the 
hand,  arm,  face,  leg  or  foot,  head  and  eyes  and  the  back. 
The  warning  may  be  unilateral  or  bilateral,  and  consist  of 
numbness,  'pins-and-needles,'  subjective  sensations  of  coldness. 

In  the  event  of  the  warning  constituting  the  whole  seizure, 
consciousness  may  or  may  not  be  temporarily  impaired.  On 
the  other  hand,  the  warning  may  proceed  to  a  further 
development  and  give  rise  to  the  incomplete  or  complete 
seizure,  as  the  case  may  be. 


566  XKRYors  DISEASES 


2.  Incomplete  attacks.     Minor  fits 

As  just  explained  the  incomplete  attack  ma}7  consist  of  the 
warning  sensation  with  a  further  development  towards  the 
complete  seizure  along  with  some  interference  with  conscious- 
ness. Hence  many  incomplete  attacks  begin  with  peripheral, 
epigastric,  cephalic,  psychical  and  special  sense  warnings. 
Many,  on  the  other  hand,  have  no  such  aura.  These  form 
an  important  type  of  minor  epileptic  seizure.  In  this 
category  are  all  those  seizures  in  which  consciousness  is 
temporarily  lost  or  impaired,  but  which  are  unaccompanied 
by  more  than  a  trifling  convulsion.  Such  are  cases  of  sudden 
fall,  *  loss  of  memory,'  '  spells,'  deviation  of  the  eyes  and 
a  cry,  pallor  of  the  face  with  deviation  of  the  head  and  a  fall, 
a  sudden  cry  accompanied  by  a  fall,  and  many  other  attacks 
of  a  similar  character.  These  attacks  are  not  uncommonly 
followed  by  a  somnambulistic-like  stage,  or  stage  of 
automatism. 

3.  Complete  attacks.     Major  fits 

The  major  attack  may  commence  with  one  of  the  warnings 
already  described,  or  with  sudden  and  complete  loss  of  con- 
sciousness. In  a  considerable  number  of  cases  the  fit  is 
ushered  in  by  a  cry,  resembling  a  screech,  which  is  produced 
during  inspiration.  The  loss  of  consciousness  precedes  or 
accompanies  the  cry.  The  patient  falls  down  in  a  state  of 
tonic  spasm,  which  is  generalised,  but  invariably  affects  one 
side  more  than  the  other.  The  head  and  eyes  are  deviated 
to  one  side  and  the  body  may  be  rotated  to  the  same  side. 
The  arms  are  abducted  at  the  shoulder,  flexed  at  the  elbow 
and  wrist,  the  fingers  being  either  extended  or  flexed.  The 
legs  are  extended  and  rotated  inwards  and  the  feet  inverted. 
The  face  becomes  livid  and  cyanosed,  and  is  drawn  over 
towrards  the  more  affected  side.  On  the  less  affected  side  the 
leg  is  extended  and  the  arm  may  be  extended  or  abducted. 
During  the  tonic  stage  respiration  is  in  abeyance  in  full 
inspiration.  The  superficial  veins  stand  out  prominently. 
This  stage  may  last  from  a  few  seconds  to  a  minute  or  a 
minute  and  a  half,  and  when  prolonged,  the  intensity  of  the 
spasm  may  pass  from  the  more  to  the  less  affected  side,  so 


EPILEPSY  567 

that  the  head  and  eyes  may  be  deviated  to  the  other  side. 
The  spasm  may  be  so  great  as  to  cause  dislocation  of  the 
shoulder  or  jaw,  accidents  which  may  readily  occur  in  succeed- 
ing seizures.  Death  may  occur  from  suffocation  in  severe 
cases  owing  to  the  face  being  buried  in  the  pillow. 

The  first  sign  of  the  yielding  of  the  tonic  stage  is  the 
development  of  a  fine  rapid  tremor.  This  rapidly  gives  place 
to  sudden,  quickly  repeated,  shock-like  contractions  of  greater 
degree,  which  become  of  larger  range  and  greater  severity, 
and  occur  at  gradually  lengthening  intervals,  and  finally 
cease  after  two  or  three  desultory  jerks.  The  limbs  and 
body  are  then  in  a  state  of  flaccidity.  Towards  the  latter 
part  of  this  stage  respiration  is  resumed— at  first  by  a  series 
of  jerky  expirations  passing  into  stertorous  breathing.  The 
duration  of  the  clonic  stage  varies,  but  is  longer  than  the 
tonic,  and  clonic  movement  may  continue  for  three  or  four 
minutes  after  the  cessation  of  the  tonic  stage. 

In  slighter  seizures  the  tonic  stage  may  be  brief,  with  fine 
tremor  persisting  throughout,  the  seizure  being  limited  almost 
entirely  to  one  side,  with  little  or  no  true  clonic  stage. 

During  the  tonic  stage  the  pupils  are  dilated  and  inactive  to 
light.  On  return  to  consciousness  they  resume  their  normal 
size,  although  if  the  fit  has  affected  one  side  more  especially 
the  pupil  on  that  side  may  remain  more  dilated  than  its 
fellow.  The  pupillary  light  reflex  may  return  during  the 
clonic  stage,  even  when  the  pupils  are  still  dilated. 

The  clonic  stage  is  followed  by  one  of  muscular  relaxation 
and  stupor,  from  which  the  patient  passes  into  deep  sleep 
and  finally  recovers. 

Accidents  accompanying  fits.  During  the  tonic  stage 
ecchimoses,  dislocations,  fracture  of  the  teeth  and  bones, 
evacuation  of  urine  and  faeces,  and  suffocation  may  occur. 
During  the  clonic  stage  tongue-biting  is  very  common,  and 
may  be  a  feature  of  some  cases. 

Sequelae  of  epileptic  fits 

If  the  patient  be  examined  immediately  after  a  severe 
fit,  a  condition  of  general  muscular  relaxation  is  observed. 
Temporary  loss  of  the  deep  reflexes  may  be  demonstrated,  but 
as  a  rule  the  deep  reflexes  are  increased  and  clonus  easily 


568  NERVOUS   DISEASES 

elicited.  The  superficial  abdominal  reflexes  are  temporarily 
abolished.  The  behaviour  of  the  plantar  reflex  is  interest- 
ing. At  first,  no  response  may  be  obtained  on  stimulation; 
later  an  extensor  response  is  obtained,  which  after  a  short 
period  gives  place  to  flexion.  Eventually  the  reflexes  become 
normal,  although  a  permanent  increase  of  the  deep  reflexes  is 
usual  in  epileptics. 

Transient  motor  and  sensory  paralyses — exhaustion  para- 
lyses-may also  be  observed.  They  are  best  seen  in  cases 
in  which  one  side  has  been  predominantly  convulsed.  The 
head  and  eyes  may  be  deviated  towards  the  less  affected  side 
with  a  slight  degree  of  motor  weakness  on  the  opposite  side. 

Sensory  phenomena  consist  of  a  slight  and  general  blunting 
of  sensibility  over  the  side  which  has  been  most  convulsed. 
This  rapidly  clears  away,  the  proximal  segments  recovering 
before  the  distal.  These  phenomena  are  comparatively  rare, 
but  occur  in  cases  in  which  no  evidence  of  gross  cerebral 
lesion  has  shown  itself  in  the  subsequent  history  of  the  case. 
The  transient  nature  of  the  symptoms  may  therefore  be  put 
down  to  a  temporary  exhaustion  rather  than  to  a  progressive 
destruction  of  the  cortical  centres. 

Amblyopia,  restriction  of  the  visual  fields,  deafness,  and 
defects  of  smell  and  taste  have  also  been  observed. 

The  immediate  psychical  effects  are  described  on  p.  569. 

The  Psychical  Element 

The  mental  conditions  found  in  epilepsy  are  :— 

1.  The  epileptic  character  and  temperament. 

2.  The  paroxysmal  psychoses  which  precede  or  succeed 

the  convulsive  phenomena. 

3.  The    psychoses    which    replace    the    convulsions- 

psychical  epileptic  equivalents. 

4.  The  permanent  interparoxysmal  mental  state. 

i.  Temperament 

In  epilepsy  it  is  usual  to  find  some  form  of  mental  ob- 
liquity. The  memory  is  usually  impaired  for  recent  events, 
and  is  often  of  a  perverted  kind.  Epileptics  are  self- 
opinionated  and  egotistical,  and  their  conversation  is  prolix 
and   pretentious.     Their   mental  perspective  is  blurred  and 


EPILEPSY  569 

disproportioned.  Their  judgment  is  feeble,  they  are  fre- 
quently credulous  and  mystical  and  given  to  superstitious 
ideas  and  fancies.  The  majority  possess  a  religious  fervour, 
which  forms  a  marked  feature  of  the  disease  and  contrasts 
strongly  with  their  actions,  their  ideas  of  right  and  wrong 
being  often  vague.  Their  want  of  initiative  is  striking, 
and  mainly  on  this  account  they  require  supervision  and 
direction. 

They  are  subject  to  moods  of  hastiness  of  temper  and 
pugnacity  alternating  with  laziness  and  lethargy,  which  make 
them  difficult  to  live  with  and  often  useless  as  workers. 
Many  show  habitual  irritability  of  temper  and  awkwardness 
of  disposition,  while  others  are  imbecile,  demented,  delusional, 
or  liable  to  dangerous  impulses. 

2.  Paroxysmal  psychoses.      Epileptic  insanity 

These  forms  of  mental  aberration  or  perversion  are 
incidental  to  the  seizures,  and  occur  either  as  prodromal 
phenomena  or  as  immediate  sequelae  of  the  attacks.  They 
include  all  the  hallucinatory,  delusional,  maniacal,  melancholic, 
and  psychasthenic  states  which  are  observed  in  the  subjects  of 
epileptic  fits. 

If  they  are  pre-paroxysmal  in  occurrence,  they  ought  to 
be  regarded  as  a  prolonged  or  modified  prodromal  stage. 

If  post -paroxysmal,  they  take  the  form  of  definite 
psychical   phenomena   lasting   for   variable   periods. 

The  commoner  post-paroxysmal  forms  are  : — 

(a)  Acute  epileptic  dementia :  a  condition  more  commonly 
observed  after  serial  or  status  outbursts,  consisting  of  profound, 
though  temporary,  mental  and  physical  debility  and  stupor. 

(b)  Acute  epileptic  mania:  a  rarer  psychical  condition  than 
the  preceding,  but  of  much  importance  on  account  of  the 
violence  of  the  psycho-motor  disturbances.  It  is  usually  brief, 
not  lasting  more  than  about  twelve  hours.  Minor  degrees  are 
talkativeness,  tiresomeness,  and  exhilaration  of  spirits. 

(c)  Transitory  delusional  states.  These  are  a  common  form 
of  post-paroxysmal  psychosis,  and  may  last  for  several  days 
before  the  patient  is  restored  to  his  normal  mental  condition. 

(d)  Automatism.  Most  seizures — but  especially  the  minor 
forms  — are  succeeded  by  some  kind  of  automatic  action,  of 
which  undressing  is  probably  the  most  common. 


570  NERVOUS  DISEASES 


3.  Psychical  epileptic  equivalents 

It  is  now  generally  conceded  that  psychical  phenomena 
may  replace  convulsive  seizures  in  some  cases  of  epilepsy. 
The  general  tendency  of  recent  writing  on  this  subject  has 
been  to  enlarge  the  scope  of  the  epileptic  manifestations, 
and  to  regard  as  epileptic  various  psychical  conditions  of  a 
periodic  nature.  In  this  way  some  psychical  states  have 
been  included  amongst  the  epileptic  manifestations,  which 
are  in  reality  common  to  all  the  psychopathies. 

Our  own  observations  confirm  the  opinion  that  no  mental 
state  occurs  as  an  epileptic  equivalent,  which  is  not  also  seen 
as  a  pre-  or  post-paroxysmal  psychosis.  We  are,  therefore, 
in  favour  of  defining  psychical  epileptic  equivalents  as  the 
mental  phenomena  of  the  pre-  and  post-paroxysmal  states, 
occurring  without  convulsion  or  spasm. 

The  following  conditions  are  seen  as  psychical  equivalents 
of  epileptic  fits  : — 

(a)  Psychical  epilepsy,  or  attacks  of  brief  duration, 
consisting  mainly  in  the  performance  of  simple  automatic 
actions.  Attacks  of  a  more  prolonged  character — ambulatory 
automatism — are  sometimes  epileptic,  but  are  more  commonly 
hysterical.  '  Wandering '  is  a  not  uncommon  feature  in 
confirmed  epilepsy,  but  is  also  seen  in  alcoholism,  dementia, 
and  congenital  mental  deficiency. 

(b)  Epileptic  mania,  resembling  in  all  respects  the  acute 
maniacal  outburst  of  the  post-paroxysmal  psychosis  is  rare, 
but  should  be  recognised.  It  has  received  the  name  of  '  grand 
mal  intellectuel.'  In  contrast  to  it  is  the  '  petit  mal  intellec- 
tuel,'  or  epileptic  impulsion — short  attacks  of  extreme 
suddenness,  in  which  the  patient  may  effect  a  homicidal  act. 

(c)  Transitory  delusional  states. 

(d)  Catatonic  stupor. 

4.  The  permanent  dementia 

The  permanent  or  inter-paroxysmal  mental  state  of  epi- 
leptics varies  from  that  in  which  little  or  no  mental  impairment 
can  be  detected  to  one  in  which  all  the  features  of  dementia 
are  observed— viz.  defective  memory,  confusion  of  ideas,  im- 
paired capacity  for  work,  absence  of  initiative,  and  a  slow  and 


EPILEPSY  "i  I 

dull  comprehension.  The  proportion  of  epileptics  in  which 
this  condition  is  present  varies ;  but  it  may  be  stated  that 
in  institutions  for  epileptics,  where  those  are  collected 
who  are  unable  to  work  owing  to  their  infirmity,  only 
about  13  per  cent,  of  the  inmates  are  without  some  mental 
deficiency. 

The  inter-paroxysmal  dementia,  although  an  integral  part 

.  of  the  disease,  is  modified  by  various  factors— such  as  a  strong 

neuropathic  family  history,  the  duration  of  the  seizures,  the 

age  at   the  onset,  and  the  frequency  and  character   of  the 

attacks. 

Clinical  Types  of  Epilepsy 

Epilepsy  is  commonly  described  and  its  clinical  varieties 
grouped  according  to  the  type,  the  frequency,  or  the  time  of 
occurrence  of  the  seizures.  If  classified  according  to  the  cha- 
racter of  the  seizures  three  main  divisions  may  be  described— 
the  major,  minor,  and  combined  types;  if  according  to  the 
periodic  frequency  of  the  seizures— serial  epilepsy,  and  the 
status  epilepticus  ;  if  according  to  the  time  of  occurrence 
—nocturnal  and  diurnal  epilepsies. 

The  major  type  of  epilepsy  is  characterised  by  the  recur- 
rence of  major  convulsive  seizures  varying  in  frequency  from 
one  or  two  up  to  twenty  or  more  per  mensem.  As  in  all 
forms  of  epilepsy  there  is  great  constancy  in  the  occurrence 
of  the  fits,  little  change  either  in  character  or  frequency  being 
observed  over  long  periods  of  time.  The  mental  condition  in 
this  type  is  not  associated  with  any  special  degree  of  dementia, 
which  is  indeed  largely  determined  by  the  frequency  of  the 
fits.  During  many  years  the  mental  condition  may  not  show 
signs  of  deterioration,  but  a  marked  increase  in  the  fits,  or 
an  attack  of  status  epilepticus  may  be  the  starting-point  of 
progressive  dementia  or  delusional  insanity. 

The  minor  type,  characterised  by  the  presence  of  minor 
seizures  only,  is  in  its  pure  form  not  common.  The  '  sensa- 
tions '  may  be  very  frequent — up  to  several  daily.  The  usual 
psychical  state  consists  of  a  mild  degree  of  mental  impairment 
—  chiefly  loss  of  memory  for  recent  events. 

The  combined  type  is  characterised  by  the  combination 
of  major   and   minor    seizures,    sometimes    associated   with 


572  NERVOl'S  DISEASES 

psychomotor  attacks.  It  is  the  type  of  epilepsy  which  shows 
the  greatest  frequency  of  iits,  sometimes  up  to  several  hundred 
in  the  course  of  a  month  or  two.  As  might  be  expected  this 
type  presents  the  most  profound  degrees  of  dementia,  and  is 
little  influenced  by  treatment. 

Serial  epilepsy  is  a  type  of  the  disease  in  which  a  series 
or  succession  of  tits  of  either  the  major  or  minor  type,  or 
of  a  combination  of  major  and  minor  attacks,  forms  the 
picture  of  the  disease.  It  is  a  subacute  form  of  epilepsy  and 
differs  from  the  status  epilepticus  by  a  more  or  less  complete 
return  to  consciousness  between  the  seizures.  It  may  occur 
as  the  type  of  the  disease,  as  a  modified  form  of  status,  or  as 
an  occasional  complication  of  the  major,  minor,  or  combined 
types.  The  serial  outburst  is  usually  heralded  by  a  pro- 
dromal increase  in  the  single  seizures,  which  increase  in 
frequency  until  a  climax  is  reached,  from  which  a  numerical 
decrease  in  frequency  occurs,  with  eventual  cessation  of  fits 
for  a  time.  This  type  gives  rise  to  temporary  mental  failure 
which  has  been  already  described,  and  not  infrequently 
terminates  in  dementia. 

Status  epilepticus  is  the  most  acute  manifestation  of 
epilepsy,  and  in  some  of  its  developments  may  form  the  type 
of  the  disease.  It  may  be  an  occasional  feature  of  all  forms  of 
epilepsy,  or  it  may  result  from  some  accidental  circumstance, 
such  as  sudden  stoppage  of  the  bromides,  emotional  shock, 
an  acute  inflammatory  disorder,  or  a  fall  or  blow  upon  the 
head. 

Its  onset  is  foreshadowed  by  the  increase  of  the  ordinary 
seizures,  which  increase  in  frequency,  until  one  attack  succeeds 
another  so  rapidly  that  consciousness  is  not  regained.  The 
attack  ends  in  coma  and  exhaustion,  in  which  the  patient 
may  die,  or  he  may  recover  after  a  short  period  of  delusional 
insanity  and  irritability. 

Status  epilepticus  is  the  most  serious  complication  of 
epilepsy,  and  although  the  prognosis  is  grave,  it  is  not 
necessarily  fatal. 

Nocturnal  and  diurnal  epilepsy.  There  is  a  type  of 
epilepsy,  in  which  the  fits  occur  only  during  sleep ;  and  it  is 
a  well  known  fact  that  in  the  combined  type  of  the  disease, 
the  minor  fits  may  occur  by  day,  and  the  major  during  the 
sleeping  hours. 


EPILEPSY 


573 


The  usual  incidence  of  epileptic  fits  is  an  irregular 
periodicity,  and  observations  have  frequently  been  made  to 
ascertain  whether  any  hour  of  the  day  or  night  especially 
favours  the  occurrence  of  seizures.  Our  own  observations 
are  recorded  in  the  annexed  chart,  which  shows  (1)  that 
fits  are  more  common  during  sleeping  than  waking  hours,  and 
(2)  that  the  fit  incidence  attains  its  maximum  during  the 
early  hours  of  deepest  sleep. 

Chart    showing    the    Hourly    Frequency   of    Fits    in 
Sixty-two  Cases  of  Epilepsy 


MIDMHT                                A.M.                            NOON                        P.M.                                   MID.NT 
12     1     2    3     4    5    S     7     8     9    10    II    12    1      2    3     4   5    6    7    8    9    10  1 1    12 

NUMBER 

OF  FITS 

35 

30 

25 

20 

15 

10 

5 

l 

\ 

\ 

s 

\ 

/ 

s 

/ 

/ 

S 

\ 

i„ 

/ 

\ 

i. 

/ 

\ 

/ 

Onset,  course,  and  termination 

Epilepsy  may  commence  in  a  variety  of  ways,  of  which 
the  following  are  the  most  frequent : — 

(a)  A3  the  minor  type,  which  may  persist  for  some  years 
before  a  major  convulsion  ensues. 

(b)  As  the  major  type  which  persists. 

(c)  As  the  nocturnal  variety,  which  may  exist  unrecognised 
for  many  years. 

(d)  By  the  occurrence  of  a  single  fit,  which  may  precede 
the  full  development  of  the  disease  by  months  or  years. 

(e)  By  an  attack  of  status  epilepticus,  occurring  during 
confinement  or  the  puerperium. 

Commencing  in  one  or  other  of  these  ways,  epilepsy  may 
persist  for  many  years.  The  frequency  of  the  seizures  varies 
enormously,  but  a  general  idea  may  be  obtained  by  reference 
to  the  following  Table,  which  shows  the  total  number  and 
percentage  frequency  of  epileptic  fits  in  300  cases  of  the 
disease,  observed  in  an  institution  for  epileptics. 


574  NERVOUS  DISEASES 

Frequency.  Total.        Percentage. 

Daily  fits                             46  15 

Weekly  fits       .  .       133  44 

Monthly  fits                        94  31 

Quarterly  fits                      12  4 

Yearly  fits                           12  4 

At  longer  intervals  .           3  1 


Total        .         .       300  99 

Long  remissions,  sometimes  lasting  for  many  years,  are 
a  not  uncommon  feature  of  epilepsy.      They  may  be  seen  : — 

(a)  During  childhood,  usually  from  about  five  to  twelve 
years  of  age,  in  those  whose  fits  commence  in  infancy.  A 
rarer  period  of  remission  extends  over  the  ages  of  puberty 
and  early  adolescence. 

(b)  After  the  complete  establishment  of  the  disease,  either 
spontaneously  or  assisted  by  treatment.  These  remissions 
may  last  from  two  up  to  thirty  years,  but  the  commonest 
period  of  freedom  lasts  about  three  or  four  years.  Freedom 
from  fits  for  more  than  four  years  is  frequently  regarded 
as  a  '  cure,'  of  epilepsy. 

Termination.  Epilepsy  is  a  progressive  disorder  ending 
either  in  a  cure  in  a  small  percentage  (approximately  ten  per 
cent.)  or  in  mental  infirmity,  delusional  insanity  or  dementia. 
The  actual  cause  of  death  is  found  in  pulmonary  complications, 
the  status  epilepticus,  accidents  occurring  during  a  fit,  organic 
heart  disease,  or  as  the  direct  consequence  of  a  fit. 

The  mean  age  at  death  is  stated  to  vary  from  thirty  to 
forty-eight  years  of  age. 

Diagnosis 

The  recognition  of  epileptic  seizures  is  based  upon  certain 
features,  one  or  more  of  which  should  be  present  before  a 
diagnosis  of  epilepsy  is  made.     These  are  : 

(a)  Sudden  loss  or  obscuration  of  consciousness.  The  exist- 
ence of  this  feature  at  some  period  is  essential  to  the  diagnosis 
of  epilepsy,  although  each  and  every  epileptic  manifestation  is 
not  necessarily  associated  with  a  loss  of  consciousness. 

(b)  A  sudden  fall  from  obscuration  of  consciousness  is  an 
epileptic  symptom,  but  many  types  of  seizure  are  unaccom- 
panied by  a  fall. 


EPILEPSY 


575 


(c)  The  sequence  of  tonic  spasm  followed  by  clonic  convul- 
sion, with  their  effects  bed- wetting  and  tongue-biting  is 
conclusive   evidence. 

Differential  Diagnosis   Between  an   Epileptic  and  an    Eystericai    Fit 


Epileptic  Fit. 

Eystkuical  Pit. 

Cause 

No  exciting  cause 

Exciting  causes  frequent 

Aura 

May  be  present 

Usually  no  aura 

Onset 

Sudden  ;    sometimes  with  a 
cry 

Gradual;  sometimes  with 
screaming 

Consciousness 

Lost 

Impaired  or  perverted 

Fall 

Sudden ;  often  causing  in- 
jury 

Gradual ;  does  not  cause 
injury 

Corneal  Reflex 

Absent 

Impaired,  sometimes  absent 

Pupil  Reflex 

Reaction  to  light  abolished 

Reaction  to  light  retained 

Motor  symptoms 

Tonic    spasm  passing  into 
clonic.       Conjugate   de 
viation  of  head  and  eyes 
to    side    more    a  fleeted ; 
movements  unaffected  by 
interference 

Tonic  spasm,  opisthotonos 
or  sudden  change  to  pros- 
thotonos.  Movements 
semi-purposive  and  co- 
ordinated. Strabismus 
divergent  or  convergent. 
Movement  increased  by 
interference. 

Micturition 

May    occur    during    tonic 
stage 

Occurs  after  fit  is  over 

Tongue-biting 

May   occur   during    clonic 
stage 

Never  occurs,  but  patient 
may  bite  her  lips  or  her 
attendants 

Post  -  convulsive 

stage 

Stupor,  drowsiness ;   some- 
times    automatism     and 
mental  confusion 

Sudden  recovery,  patient 
starts  up  and  asks  what 
has  happened.  No  auto- 
matism or  mental  con- 
fusion 

Reflexes 

Transient  reflex  changes  as 
in    disturbance    of    the 
pyramidal  system 

No  changes  of  an  organic 
nature 

576  NERVOUS  DISEASES 

A  differential  diagnosis  has  to  be  made  from  : — 

1.  Hysterical  seizures. 

2.  Focal  epilepsy. 

3.  Attacks  associated  with  labyrinthine  disease. 
■4.  Cardiac  syncopal  attacks. 

5.  '  Vagal '  attacks. 

1.  Epileptic  and  hysterical  attacks.  The  epileptic  fit 
pursues  a  well-defined  course,  and  is  followed  by  a  series  of 
immediate  sequela).  In  the  hysterical  seizure  no  such  course 
and  sequelae  are  observed.  The  rolling  or  squinting  move- 
ments of  the  eyeballs,  and  the  oscillations  of  the  head  in 
hysteria,  are  quite  distinct  from  the  tonic  conjugate  deviation 
of  the  head  and  eyes  in  epilepsy.  The  tonic  spasm  of  the 
limbs  in  epilepsy  contrasts  with  the  tonic  spasm  of  the  neck 
and  trunk  in  hysteria.  The  steady  evolution  and  eventual 
cessation  of  the  clonic  convulsions  of  epilepsy  are  quite 
distinct  from  the  vibratory  tremors  of  the  hysterical  seizure. 
The  sudden  relaxation  of  spasm  and  the  almost  instantaneous 
return  to  consciousness  in  the  latter  are  noteworthy  features. 

The  mam  points  in  differential  diagnosis  are  given  in  the 
Table  on  p.  575. 

2.  Epilepsy  and  focal  epilepsy.  In  focal  epilepsy  con- 
sciousness is  preserved.  The  fit  commences  with  clonic 
convulsions  in  a  particular  locality,  and  spreads  from  its 
seat  of  origin  by  a  definite  march.  In  severe  cases  the 
convulsion  may  become  generalised  with  abolition  of  con- 
sciousness. In  doubtful  cases  an  examination  of  the  motor, 
sensory,  and  reflex  phenomena  will  usually  permit  of  a 
diagnosis  being  made. 

3.  Minor  epilepsy  and  aural  vertigo.  The  chief  points 
of  resemblance  and  difference  are  seen  in  the  following 
Table  :— 

Minor  epilepsy.  Aural  vertigo. 

Onset  sudden.  Onset  sudden. 

Duration  brief.  Duration  brief. 

Temporary  loss  of  conscious-    Temporary  loss  of  conscious- 
ness, ness  sometimes  present. 
Fall,  more  or  less  sudden.           Fall,  usually  with  a  feeling  of 

being  forced  or  dragged  down. 


EPILEPSY  577 

Minor  epilepsy.  Aural  vertigo. 

Frequently    post -paroxysmal  Never  automatism. 

automatism. 

No  local  signs  of  ear  disease.  Tinnitus    and    labyrinthine 

deafness. 

Inter-paroxysmal  mental  state  No  impairment  or  dementia, 
as  in  epilepsy. 

4.  Epilepsy  and  cardiac  syncope.  It  is  only  in  the 
minor  type  of  epilepsy  that  a  difficulty  is  likely  to  arise. 
The  points  in  the  differential  diagnosis  are : — 

Minor  epilepsy.  Cardiac  syncope. 

Loss  of  consciousness  sudden  Loss  of  consciousness  gradual 

and  complete.  and  deliberate. 

Pallor   and    flushing    of    the  Marked  pallor  of  face,  and  a 

face  rare.  general  feeling  of  coldness. 

Pulse  not  affected.  Feeble  action  of  pulse. 

Post-paroxysmal   automatism  Never    confusion     after     the 

and  confusion.  attack. 

5.  Minor  epilepsy  and  '  vagal '  attacks.  The  following  are 
the  features  of  the  '  vagal '  attack  as  given  by  Sir  W.  Gowers 1 : 
(a)  the  aura  is  referred  to  the  abdominal,  cardiac,  or 
respiratory  organs  ;  (6)  the  sensation  ascends  to  the  chest, 
throat,  and  head  ;  (c)  it  is  accompanied  by  a  feeling  of 
cardiac  or  respiratory  distress,  of  fear  or  of  impending  death  ; 
(d)  there  is  no  true  loss  of  consciousness,  but  sometimes 
a  feeling  of  unreality  ;  (e)  the  attack  terminates  with 
acceleration  of  the  heart's  action  ;  (/)  it  lasts  about  fifteen 
or  twenty  minutes. 

Prognosis 

Epilepsy  is  a  chronic  disease,  and  in  the  majority  of 
cases  tends  towards  increasing  frequency  of  the  seizures  and 
mental  deterioration.  A  considerable  percentage  of  cases,  as 
a  result  of  carefully  considered  and  well  sustained  treatment 
over  a  number  of  years,  result  in  improvement  or  recovery. 

The  following  facts  have  to  be  taken  into  consideration  in 
estimating  the  prognosis. 

1  Gowers,  Sir  W.,  The  Borderland  of  Epilepsy,  1907. 

37 


578  NERVOUS  DISEASES 

J.  A    neuropathic    family   history    does    not    necessarily 

stand  in  the  way  of  arrest  of  the  disease,  or  of  its  improve- 
ment under  treatment. 

2.  The  age  at  onset  is  important.  If  arising  under  ten 
years,  the  outlook  is  unfavourable  ;  if  arising  during  puberty, 
the  chances  of  improvement  or  recovery  are  greater.  Senile 
epilepsy,  or  that  arising  over  fifty  or  fifty-five  years  of  age, 
is  a  tractable  disorder. 

3.  The  duration  of  the  disease  influences  the  prognosis  to 
the  extent  that  the  earlier  a  patient  comes  under  treatment 
the  more  hopeful  is  the  outlook.  Fits  may  be  arrested  even 
after  a  duration  of  twenty  or  thirty  years. 

4.  The  frequency  and  character  of  the  fits.  Major  seizures 
occurring  at  long  intervals  present  the  most  favourable  form 
of  epilepsy.  Fits  occurring  daily  or  weekly  are  unfavour- 
able, especially  when  of  the  combined  type.  Minor  seizures, 
especially  when  associated  with  slight  degrees  of  mental 
impairment,  are  unsatisfactory  to  treat. 

5.  Marked  mental  impairment  is  an  unfavourable  feature; 
epileptics  of  a  degenerative  type  are  also  unfavourable. 

A  cure  or  permanent  arrest  of  fits  takes  place  in  from  ten 
to  twelve  per  cent,  of  the  cases  of  epilepsy,  which  have 
persisted  with  treatment  over  prolonged  periods. 


Treatment 

In  the  treatment  of  epilepsy  two  factors  have  to  be  con- 
sidered :  (1)  the  inherent  instability  of  the  nervous  system, 
and  (2)  the  various  conditions  and  circumstances  which  may 
excite  or  aggravate  the  disease.  The  first  factor  is  invariably 
present  in  greater  or  less  degree,  and  in  most  cases  careful 
examination  reveals  one  or  more  exciting  causes.  Every  case 
ought  therefore  to  be  studied  systematically ;  and  the  impor- 
tance of  maintaining  the  general  health  of  the  patient  cannot 
be  too  strongly  insisted  upon.  In  many  instances  the 
instability  of  the  nervous  system  per  sc  is  slight,  and  pro- 
vided that  exciting  causes  can  be  removed  very  little  sedative 
treatment  is  sufficient  to  keep  the  patient  free  from  all 
manifestations  of  the  disease. 

Some  of  the  more  common  of  the  exciting  or  reflex  causes 
of  epilepsy  are  worthy  of  special  mention  : — 


UPILEPSY  570 

Alimentary  system.  Diseased  teeth,  pyorrhrea,  gastric 
disturbance,  constipation,  and  worms. 

Circulatory  system.  Low  or  high  blood  pressure,  feeble 
circulation,  an  unstable  vaso-motor  system,  and  anaemia. 

Reproductive  system.  Sexual  excitement  and  irregularity 
in  the  menstrual  functions  in  women. 

.  Attention  ought  also  to  be  paid  to  the  general  hygiene  of 
the  patient  as  regards  exercise,  fresh  air,  regular  mode  of  life, 
and  the  avoidance  of  excitement  or  emotional  stress. 

Medicinal  treatment.  The  salts  of  bromine  are  the  most 
useful  remedies  in  the  treatment  of  epilepsy.  They  may  be 
given  in  various  forms — such  as  the  bromides  of  potassium, 
sodium,  ammonium,  or  strontium.  Of  these  salts,  potassium 
bromide  is  probably  the  most  efficient.  Sodium  bromide  may 
be  substituted  in  the  less  severe  cases.  Ammonium  bromide 
has  been  recommended  in  cases  where  the  patients  are 
depressed — more,  however,  on  theoretical  than  on  practical 
grounds.  Strontium  bromide  has  less  tendency  to  produce 
acne  than  the  other  salts  of  bromine,  and  may  therefore  be 
given  in  cases  where  this  complication  is  troublesome.  The 
bromides  may  (1)  arrest  the  fits  permanently,  (2)  arrest  the 
fits  temporarily,  (3)  lessen  the  frequency  or  severity  of  the 
fits,  or  (4)  exercise  no  apparent  effect. 

Borax  (sodium  biborate)  is  of  no  special  value  when  given 
alone,  but  is  sometimes  serviceable  when  combined  with 
bromide,  especially  in  cases  of  minor  epilepsy.  The  dose  may 
vary  from  ten  to  twenty  grains. 

Oxide  of  zinc  in  doses  of  one  to  two  grains  once  daily  is 
sometimes  beneficial  in  cases  of  minor  epilepsy.  An  objection 
to  its  use  is  that  it  is  apt  to  cause  vomiting. 

Belladonna  is  often  successful  in  the  treatment  of  minor 
epilepsy.  It  is  prescribed  in  the  form  of  the  tincture  and 
may  be  given  in  doses  of  three  to  five  minims  combined  with 
bromide.  In  some  cases  of  minor  epilepsy,  in  which  the 
bromides  fail,  the  administration  of  belladona  alone  is 
attended  with  success. 

Digitalis,  strophanthus,  strychnine,  and  mix  vomica  are 
specially  useful,  when  combined  with  bromide,  in  those  cases 
of  epilepsy  in  which  the  fits  only  occur  during  sleep  or  in 
which  there  is  a  low  blood  pressure. 

Nitro-glycerine  has  been  advocated  in  cases  in  which  arterial 

37  * 


580  NERVOUS  DISEASES 

spasm  precedes  the  onset  of  the  tits.  In  such  cases  it  may  be 
successfully  employed,  but  it  should  not  be  prescribed  unless 
the  indications  for  its  use  are  clearly  established. 

Arsenic  is  often  prescribed,  but  has  no  special  effect  on 
epilepsy.  It  may  act  as  a  tonic,  however,  and  is  useful  in 
combination  with  the  bromides,  as  it  reduces  the  tendency  to 
acne  and  bromide  rash. 

Iron  may  be  given  in  association  with  bromide  in  cases 
with  anaemia  ;  but  it  must  be  remembered  that  in  a  small 
number  of  cases  iron  tends  to  increase  the  severity  of  the  fits. 

Method  of  administration.  The  choice  of  remedies,  the 
dose  and  the  time  of  administration  will  vary  according  to 
the  requirements  of  each  case.  In  many  instances  the  best 
results  can  only  be  obtained  by  practical  observation  of  the 
effect  of  different  forms  of  treatment.  The  following  are  the 
more  important  points  to  be  considered  before  commencing 
treatment  :  (1)  the  character  of  the  malady,  whether  the 
major,  minor,  or  combined  type;  (2)  the  frequency  of  the 
seizures  ;    (3)  the  time  at  which  the  seizures  occur. 

1.  Character  of  the  malady.  In  major  epilepsy,  bromide, 
in  doses  of  fifteen  to  twenty-five  grains  combined  with 
arsenic,  is  the  most  successful  remedy.  In  minor  epilepsy 
bromide  should  be  given  in  small  or  moderate  doses— ten  to 
twenty  grains  in  combination  with  tincture  of  belladonna 
(two  to  five  minims)  or  with  borax  (ten  to  twenty  grains)  ;  if 
there  is  circulatory  disturbance  digitalis,  strychnine,  nux 
vomica,  or  nitro-glycerine  may  be  combined  with  the  bromides, 
according  to  the  indications  present  in  each  case.  Zinc  oxide 
is  useful  in  some  cases.  In  combined  epilepsy  bromide  may 
be  given  alone  or  in  association  with  other  drugs. 

2.  Frequency  of  the  seizures.  The  amount  of  bromide 
ought  to  be  varied  in  proportion  to  the  frequency  of  the  fits. 
If  the  fits  only  occur  once  in  every  two  or  three  weeks,  a 
daily  dose  of  ten  to  fifteen  grains  will  probably  be  sufficient 
to  control  or  overcome  the  fits.  If  the  attacks  are  more 
frequent  the  bromide  must  be  given  more  often  (twice  or 
thrice  daily). 

3.  Time  of  occurrence.  If  the  fits  occur  only  at  night,  a 
twenty  to  thirty  grain  dose  of  bromide  should  be  given  at 
bedtime ;  and  if  there  is  any  evidence  of  a  low  blood  pressure, 
digitalis  or  nux  vomica  may  be  added  with  great  advantage. 


EPILEPSY  581 

If  the  fits  occur  immediately  after  the  patient  rises  in  the 
morning,  the  medicine  should  be  taken  half  an  hour  before 
rising.  If  the  fits  occur  at  irregular  intervals  the  medicine 
should  be  given  three  times  daily  in  moderate  doses.  In 
cases  of  serial  or  periodic  epilepsy,  the  dose  should  be 
increased  to  anticipate  the  attacks,  and  continued  in  lessened 
amount  during  the  intervals. 

Bromism.  Some  patients  are  specially  susceptible  to  the 
action  of  bromide  and  its  injudicious  use  may  give  rise  to 
toxic  symptoms  known  as  '  bromism.'  In  this  state  the 
mental  faculties  are  blunted,  the  memory  is  impaired,  apathy 
and  listnessness  induced,  the  speech  is  slow,  the  tongue 
tremulous,  and  saliva  dribbles  from  the  mouth.  The  gait  is 
often  staggering  and  the  limbs  weak.  An  eruption  of  acne 
frequently  covers  the  skin. 

Such  cases  are,  however,  extremely  rare.  A  more  common 
error  in  the  treatment  of  epilepsy  lies  in  not  giving  sufficient 
bromide,  or  in  cutting  off  the  drug  too  soon  or  too  abruptly. 
To  stop  the  administration  of  bromide  suddenly  in  patients 
who  have  been  taking  it  for  long  periods  is  fraught  with 
grave  danger  and  serious  risk.  In  most  cases  the  fits  return 
with  renewed  violence,  and  in  not  a  few  instances  status 
epilepticus  has  been  induced.  Many  cases  of  epilepsy  would 
be  cured  if  the  taking  of  a  small  dose  of  bromide  once  daily 
were  made  imperative  for  two  or  three  years  after  the  fits 
had  ceased. 

General  hygienic  methods  should  be  prescribed  along 
with  medicinal  treatment.  It  is  sometimes  better  to  treat 
even  an  early  case  of  epilepsy  in  an  institution,  or  under 
the  care  and  supervision  of  a  well-trained  nurse-attendant. 
If  fits  are  few  in  number,  the  schooling  or  other  mental 
instruction  should  not  be  stopped.  If  they  are  frequent,  or 
if  the  mental  condition  is  defective,  instruction  should  be 
carried  out  either  in  a  special  school  or  under  private  tuition 
at  home.  Field  exercises  and  games,  ordinary  amusements 
and  recreation  should  not  be  interdicted.  Massage  and  spinal 
douches  are  often  of  use  in  the  promotion  of  the  circulation, 
which  is  notoriously  sluggish.  Indoor  hospital  treatment 
is  unsatisfactory,  except  in  cases  of  great  debility,  or  when 
the  fits  are  frequent — as  in  serial  epilepsy  and  the  status 
epilepticus. 


582  NERVOUS  DISEASES 

Dietetic  treatment  is  often  a  useful  auxiliary  to  medicinal 
means.  .  In  all  cases  the  diet  should  be  such  as  can  be  easily 
digested,  and  the  meals  taken  at  regular  hours.  In  some 
cases  a  special  diet  may  be  prescribed  with  advantage. 
Vegetable  diet,  '  salt  starvation,'  and,  above  all,  a  purin-free 
diet,  permit  the  amount  of  bromide  salt  being  reduced  to 
a  minimum.  Alcohol  and  malt  extracts  tend  to  aggravate 
the  disease,  and  should  be  avoided  in  all  cases.  Tobacco 
may  be  permitted  to  a  moderate  extent. 

Outdoor  employment  in  robust  persons  is  especially  recom- 
mended ;  but  for  the  physically  frail  and  feeble  some  lighter 
form  of  work  is  desirable. 

Status  epileptic  us.  If  a  gradual  increase  in  the  number 
of  fits  suggests  the  onset  of  status  epilepticus,  the  dose  of 
bromide  salt  should  be  doubled,  and  ten  or  fifteen  grains 
of  chloral  hydrate  added  and  prescribed  every  four  or  six 
hours.  During  the  height  of  a  status  attack  nothing  will 
arrest  the  seizures  except  the  inhalation  of  chloroform. 
Hydrobromate  of  hyoscine  (-^  gr.  to  -g-V  gr.)  may  be  of 
temporally  use.  In  the  after  stage  of  stupor  careful  nursing, 
abundance  of  light  nourishment  and  tonics  are  essential. 

Acute  mania.  The  patient  should  be  protected  from 
injuring  himself  or  others.  Resort  may  be  had  to  a  padded 
room,  or  other  form  of  restraint.  No  drug  is  more  satis- 
factory than  the  injection  hypodermically  of  yt>  grain 
or  ^  grain  of  hydrobromate  of  hyoscine. 

It  is  an  old  observation  that  a  fit  commencing  with 
a  peripheral  warning  in  the  hand  may  be  arrested  by 
the  application  of  a  strap  round  the  wrist  or  arm. 
Pressure  over  the  pit  of  the  stomach,  a  draught  of  cold 
water,  or  of  sal  volatile,  has  been  known  to  arrest 
seizures  with  epigastric  warnings.  Some  patients  by  a 
form  of  auto  -  suggestion,  or  the  bringing  to  bear  a 
strong  determination,  have  succeeded  in  overcoming  the 
attack.  The  inhalation  of  nitrite  of  amyl  is  especially 
valuable  in  aborting  those  attacks,  which  give  a  warning 
of  their  onset. 

During  a  fit,  the  patient  should  be  laid  on  the  floor. 
Anything  likely  to  constrict  the  neck  should  be  removed. 
Tongue-biting  may  be  prevented  by  inserting  a  cork  between 
the  jaws.     The  post-paroxysmal  sleep  should  be  encouraged. 


EPILEPSY  583 

Epilepsy  in  Association  with  Organic  Disease 

Epileptic  seizures,  indistinguishable  from  those  of  idio- 
pathic epilepsy,  are  seen  in  association  with  the  following 
conditions : — 

1.  Cerebral  diplegia  and  infantile  hemiplegia. 

2.  Intracranial  tumour. 

3.  Cerebral  syphilis  and  general  paralysis. 

4.  Traumatic  lesions  of  the  brain. 

5.  Hydrocephalus. 

6.  Vascular  lesions — senile  epilepsy. 

In  certain  cases  the  physical  signs  of  gross  organic  disease 
of  the  brain  are  present ;  but  in  many,  such  signs  are  either 
absent  or  late  in  appearing.  The  association  of  epilepsy  with 
any  one  of  the  above  conditions  may  not  depend  upon  the 
direct  action  of  the  lesion,  but  upon  indirect  effects — such  as 
general  instability  of  the  nervous  system,  or  an  alteration  of 
the  normal  physiological  conditions. 


I\\UT    XIX 

THE   TICS 

(SYX.  :    HABIT    SPASM;     HABIT    CHOREA.       FRENCH 'MALADIES 

DES    TICS  '  ) 

A  tic  may  be  denned  as  a  'co-ordinated,  systematised, 
purposive  act,  reproducing  in  an  involuntary  manner  the 
co-ordinated  movements  of  every-day  life  '  (Miege).1  Such 
movements  may  be  initiated  by  a  definite  peripheral  irri- 
tation. Voluntary  repetition  of  the  movement  in  the  early 
stages  may  eventually  become  automatic  and  involuntary 
long  after  the  original  exciting  cause  has  yielded  to  treat- 
ment and  disappeared.  For  example,  a  foreign  body  upon 
the  cornea  leads  reflexly  to  blinking  movements  of  the 
eyelids,  which  may  be  continued  as  a  '  blinking  tic '  after 
the  removal  of  the  irritation  or  the  subsidence  of  con- 
junctivitis. 

In  normal  and  mentally  stable  persons,  reflexly  induced 
movements  do  not  lead  to  an  involuntary  and  automatic 
repetition  ;  this  only  occurs  when  the  psychical  condition  is 
unstable  or  deficient. 

Although  the  psychical  stigmata  may  be  well  marked,  the 
mental  qualities  associated  with  them  may  be  highly  developed 
along  certain  lines,  leading  in  many  cases  to  brilliancy,  but 
frequently  also  to  eccentricity.  History  records  numerous 
instances  of  tic  movements  which  were  outstanding  charac- 
teristics of  many  of  the  world's  great  men. 

In  addition  to  the  involuntary  and  automatic  movements 
mentioned  as  characteristic  of  the  tics,  there  is  also  a  form  of 

1  Miege  and  Feindel,  Tics  and  tlieir  Treatment  (Translated  by  Dr.  S.  A.  K. 
Wilson),  1907. 

58 ! 


THE  TICS 


585 


psychical  tic,  dependent  upon  idea.  These  are  the  obsessive 
tics,  which  form  a  troublesome  type  of  the  malady,  and  may 
or  may  not  be  accompanied  by  motor  equivalents.  Thus  we 
find  persons  who  must  carry  out  certain  actions  in  a  definite 
way ;  others  who  are  impelled  to  do  certain  acts  for 
fear  of  something  happening  if  they  do  not.  Of  similar 
character  is  the  dread  of  touching  certain  objects  (myso- 
phobia),    and    an    irresistible    impulse    to    count    a    certain 


Fig.  184. 


Fig.  1S5. 


Figs.  184  and  185. — Two  figures  illustrating  tic  movements  of  the 
head  and  neck. 


number  before  doing  anything  (arithmomania),  or  the 
necessity  to  find  certain  words,  which  have  to  be  repeated 
(onomatomania) . 

Etiology.  Tics  may  arise  at  airy  age  after  the  years 
of  early  childhood.  They  most  commonly  begin  during 
youth. 

They  affect  the  sexes  about  equally.  Heredity  plays  a 
very  important  part  in  their  causation.  In  the  parents  or 
collaterals,  neuropathic  maladies  are  noted — such  as  hysteria, 
alcoholism,  epilepsy,  chorea,  tics,  and  organic  nervous  diseases. 
On  the  other  hand,  mental  precocity,  brilliancy,  and  eccentricity 
may  also  be  observed. 

Infective  disorders  have  been  known  to  both  increase  and 


B86  NERVOUS  DISEASES 

to  lessen  tics  when  developed.  Mimicry  and  imitation  in  the 
young  may  be  factors  in  their  causation.  Brain  fag,  over- 
work, worry,  and  anxiety  are  likely  to  favour  their  develop- 
ment in  adults. 

VARIETIES  OF   MOTOR  TIC 

(a)  Facial  tics  are  seen  in  blinking  movements  of  the 
eyelids,  smiling,  grimacing,  mimicry,  winking,  tonic  closure 
of  the  eyelids,  and  smacking  movements  of  the  lips. 

(b)  Tics  of  the  jaws  are  observed  in  teeth  grinding, 
abnormal  movements  of  the  lower  jaw,  and  trismus  ;  also  as 
part  of  more  complicated  actions — such  as  biting  the  lips 
(cheilo-phagia)  and  biting  the  nails  (onycho-phagia). 

(c)  Tics  of  the  neck  are  characterised  by  movements  of 
jerking  and  tossing  of  the  head,  elevation  or  depression  of  the 
chin,  rotation  and  inclination,  and  movements  of  affirmation 
and  negation. 

Torticollis  may  be  regarded  in  many  cases  as  a  tic  of  the 
neck  muscles  (p.  590). 

(d)  Tics  of  the  upper  limb  are  common,  and  consist 
of  elevation,  depression,  and  twitchings  of  the  shoulder; 
abduction  and  adduction  movements  of  the  arm  ;  shrugging 
of  the  shoulders,  beating  and  striking  the  chest,  and  scratching 
movements  of  the  hands. 

(e)  In  the  lower  limbs,  knocking  or  rubbing  one  leg  against 
the  other,  tics  of  gait,  kicking,  genuflexion  and  various  com- 
plicated actions  of  a  characteristic  kind  are  seen. 

(/)  Tics  of  the  digestive  system  are  found  in  clicking 
movements  of  the  soft  palate,  continuous  swallowing  of  saliva, 
constant  eructations,  air  swallowing  and  its  associated 
phenomena  (aero-phagia). 

(g)  Respiratory  tic3  are  seen  in  sniffing,  snoring,  whistling, 
blowing,  coughing,  sobbing,  and  hiccoughing. 

(h)  Tics  of  speech  are  seen  in  the  emission  of  words  or 
phrases  without  any  relation  to  what  is  being  said,  and 
during  periods  of  silence.  Under  this  heading  the  irresistible 
utterance  of  oaths,  or  obscene  language  (coprolalia),  the 
repetition  of  words  or  sounds  (echolalia),  and  the  imitation  of 
gestures  (echokinesis)  may  be  included.  These  last  conditions 
are  more  usually  found   in  a  special  type  of  the  malady— 


THE  TICS  587 

convulsive    tic,   or    Gilles    de   la   Tourette's    disease — which 
requires  a  separate  mention. 

Convulsive  tic.  This  is  a  form  of  tic — rarely  seen  in  this 
country — characterised  by  spasmodic  jerking  movements,  with 
which  are  associated  uncontrollable  utterances,  impulsions, 
and  obsessions  dependent  upon  imperative  ideas. 


Fig.  186. — Illustrates  the  appearance  of  the  face  in  a  case  of 
extensive  tic-like  spasm  of  the  facial  muscles. 

The  spasmodic  movements  are  more  generalised  and 
bizarre  than  in  the  simple  tics  previously  described.  The 
exclamatory  utterances  consist  in  the  emission  of  words, 
sounds,  or  phrases  entirely  irrelevant  and  sometimes  blas- 
phemous ;  in  the  repeating  of  names,  sounds,  or  unfamiliar 
words,  and  the  imitation  of  gestures.  These  patients  suffer 
from  imperative  ideas  and  obsessions,  and  present  the  typical 
features  of  the  psychical  foundation  of  the  tics  to  an 
exaggerated  extent. 


588  NERVOUS  DISEASES 

This  variety  of  the  disease  is  probably  the  same  as  that 
described  in  other  countries  under  different  names— such  as 
'  Jumpers  '  of  the  state  of  Maine,  the  '  latah  '  of  the  Malays, 
and  the  '  myriat  chit '  (play  the  fool)  of  Siberia. 

Facial  spasm.  This  is  a  condition  more  properly  de- 
scribed under  the  tics,  in  which  spasmodic  Switchings  are 
limited  to  the  distribution  of  the  seventh  nerve  upon  one  side. 
The  spasms  are  of  a  clonic  character,  and  come  on  in  attacks 
lasting  from  several  minutes  or  some  hours,  with  intervening 
periods  of  comparative  freedom.  They  may  involve  all 
portions  of  the  facial  musculature,  or  be  mainly  limited  to 
the  orbicularis  palpebrarum,  the  angle  of  the  mouth,  and  the 
chin  muscles. 

The  cause  may  be  found  in  a  peripheral  irritation  in  the 
distribution  of  the  fifth  nerve — such  as  the  nose,  eyes,  mouth, 
or  teeth.  In  the  majority  of  cases  no  such  cause  is  detected ; 
or,  if  at  one  time  such  exciting  cause  was  present,  it  has  long 
since  ceased  to  act. 

The  disease  has  a  prolonged  course,  usually  persisting 
throughout  the  remainder  of  life.  It  is  not  painful.  It  is 
more  common  in  women  than  in  men,  and  treatment  is  of 
little  avail. 

The  only  condition  with  which  this  is  likely  to  be  confused 
is  a  localised  or  epileptiform  convulsion  arising  from  cortical 
irritation  of  the  face  area. 

Diagnosis.  As  already  denned,  the  diagnosis  of  tic  is 
based  upon  a  coexistent  motor  and  mental  association. 
There  are,  however,  numerous  disturbances  of  motility — 
such  as  spasm,  chorea,  cramp-like  movements,  myoclonus, 
mannerisms,  and  stereotyped  acts,  which  it  is  important 
should  be  distinguished  from  tic. 

This  is  often  no  easy  matter,  but  certain  general  guiding 
lines  may  be  laid  down. 

(a)  Tic  movements  are  under  the  influence  of  the  will  to 
the  extent  that  they  can  be  controlled  temporarily,  but  their 
repression  is  accompanied  by  mental  distress. 

(b)  Distraction  of  the  mind  lessens,  fatigue  and  mental 
emotion  increase  the  activity  of  the  movements. 

(c)  Tics,  as  a  rule,  disappear  during  sleep. 

(d)  Tics  are  associated  with,  and  found  exclusively  in, 
those  who  present  certain  definite  mental  peculiarities. 


THE  TICS 


589 


The    chief  points   of   differential    diagnosis   may    best    be 
reproduced  in  the  form  of  a  table. 


Table  giving  the  Chief  Points  of  Differential  Diagnosis 


Tic. 

Clonic 
Spasm. 

Chorea. 

Paramyoclonus 
Multiplex. 

Stereo- 
typed Act. 

Checked 

or  modified, 
but      with 
mental  dis- 
tress 

Influence 
of  volition 

Controlled 
temporarily 
with  great 
distress 

No  effect 

Slight  increase 
or   no  effect 

None 

Type  of 
movement 

Co-ordinate, 
purposive, 
repeated 

Abrupt,     in- 
co-ordinate, 
limited    to 
definite  area 

Erratic, 
irregular, 
not  repeated 
in         similar 
fashion 

Muscular 
fascicular 
contractions, 
instantaneous, 
involuntary, 
bilateral 

Mannerisms 

of      multi- 
form   type 
and      cha- 
racter 

Influence 
of  sleep 

Usually 
arrested 

Not  arrested 

Arrested 

Continued 
during  sleep  as 
a  rule 

Arrested 

Associated 
symptoms 

Obsessions. 
Mental  fea- 
tures as  de- 
scribed   in 
text 

Possibly 
signs  of 
organic 
disease 

Pains, 
perhaps     ar- 
thritis     and 
endocarditis 

Increase  of  myo- 
tatic  irritability 
Legs      usually 
most  affected 

None 

Course  of 
the  disease 

Usually 
persistent, 
sometimes 
incurable 

Depends    on 
cause 

Recovery  usual 

Incurable 

Eecovery 
possible 

Prognosis,  Taken  as  a  whole,  the  outlook  as  regards 
recovery  in  cases  of  tic  is  unfavourable.  In  young  patients, 
however,  suitable  treatment  directed  more  especially  to  the 
correction  of  the  movements  by  hygienic  and  educational 
methods,  may  bring  about  a  satisfactory  and  sometimes 
permanent  arrest.  When  they  start  in  adults,  on  the  other 
hand,  or  if  the  movements  have  been  in  existence  for  long 
periods,  the  disease  is  likely  to  become  permanent.  In  the 
severer  forms  with  pronounced  mental  symptoms — such  as 
the  convulsive  tics — the  prognosis  is  grave ;  for  insanity 
may  develop,  and  the  persistence  of  the  motor  disturbances 
and  severe  obsessional  feeling  may  lead  to  suicide. 

Treatment.  All  ordinary  methods  of  treatment  are  of 
little  use  in  this  disease. 

Medicinal  remedies— such  as  the  bromides,  valerian, 
cannabis  indica,  belladonna,  gelsemium,  and  arsenic  may,  one 
and  all,  be  of  some  temporary  value.     A  useful  combination 


590  NERVOUS  DISEASES 

has  been  found  in  a  mixture  containing  bromides,  cannabis 
indk-a.  and  conram. 

Massage,  hydrotherapeutics,  and  the  application  of  elec- 
tricity are  also  of  little  more  than  transient  value.  Certainly 
some  forms  of  torticollis  are  made  worse  by  the  galvanic 
current. 

Suggestion  is  of  little  use  in  cases  of  true  tic.  In  cases  of 
hysterical  spasm,  or  in  those  tic-like  conditions  associated 
with  hysteria,  hypnotic  suggestion  is  of  real  benefit,  and  is 
sometimes  effectual  in  promoting  a  cure. 

The  only  certain  and  satisfactory  treatment  of  tic 
movements  lies  in  the  persistent,  prolonged,  and  regular 
execution  of  special  movements  of  the  affected  muscular 
groups.  By  this  means  both  the  mental  and  physical 
elements  of  the  malady  are  corrected.  The  treatment  rests 
generally  upon  the  principle  of  providing  exercises,  which,  in 
the  first  place,  tend  to  arrest  the  abnormal  movements,  and, 
secondly,  counteract  the  abnormal  by  normal  movements. 
On  the  one  hand,  the  patient  is  encouraged  to  restrain 
the  movements — at  first  for  short  periods,  and  later  for 
longer  periods — in  all  positions  and  attitudes  :  from  those  of 
greatest  ease  and  comfort  up  to  those  which  involve  walking 
and  talking.  On  the  other  hand,  and  concurrently,  the 
patient  is  instructed  in  the  execution  of  slow,  regular, 
systematic,  and  accurate  movements  of  the  muscles  involved 
in  the  tie. 

These  seances  should  at  first  last  only  a  few  minutes,  and 
later  be  increased  up  to  half  an  hour  several  times  daily. 
They  should  be  carried  out  before  a  mirror,  so  that  irregu- 
larities and  mistakes  may  be  seen  and  corrected.  They 
recpjire  to  be  continued  for  long  periods,  even  after  the  tic  has 
apparently  subsided. 

Although  'habits'  in  children  are  not  tics,  they  should 
be  carefully  handled  by  judicious,  moral,  and  systematic 
treatment. 

TORTICOLLIS  TIC 

Putting  aside  the  cases  of  '  rheumatic  stiff-neck,'  spas- 
modic torticollis  is  the  most  common  variety  of  wry-neck.  It 
is  one  of  the  manifestations  of  tic. 


THE  TICH 


591 


In  the  early  stages  of  the  disease  a  movement  of  the  head 
is  present — at  first  temporary  and  controllable ;  but  later  on, 
ancl  in  severer  cases,  the  deviation  of  the  head  may  become 
persistent  without  relaxation,  and  associated  with  spasmodic 
movements  of  other  muscles. 

Etiology.  It  is  more  common  in  men  than  in  women. 
It  occurs  mainly  in  adult  life.  A  neurotic  heredity  is  found 
in  most  cases,  and  the  disease  frequently  develops  in  those 
who  have  exhibited  ner- 


vous  or  hysterical  ten- 
dencies. It  may  follow 
upon  injury,  sudden 
movement  of  the  head, 
or  in  consequence  of 
pain. 

Symptoms.  The  on- 
set of  the  spasmodic 
movements  is  gradual 
over  several  weeks  or 
months.  In  the  early 
stages  there  may  be  a 
temporary  and  complete 
cessation,  but  once  de- 
veloped the  progress  is 
usually  steady. 

In  whatever  manner 
it  starts,  three  funda- 
mental features  are 
demonstrable;  first,  the 
movements  are  such  as  can  be  voluntarily  imitated ;  secondly, 
in  the  early  stages,  the  movements  are  to  a  large  extent  under 
voluntary  control ;  and,  thirdly,  the  movements  are  the  con- 
sequence of  an  uncontrollable  impulse. 

If  traced  to  its  origin,  it  is  found  that  in  the  initial  stages 
a  desire  to  perform  a  movement  is  present.  The  mental 
impression  of  the  patient  is  that  if  the  movement  is  carried 
out  mental  satisfaction  will  result.  The  result  is,  however, 
the  reverse,  as  dissatisfaction  and  remorse  follow  in  its 
wake.  If  the  movement  is  controlled  by  an  effort  of  the 
will,  great  distress  ensues.  These  are  the  features  common 
to  all  tics. 


Fig.  187. — Shows  the  appearance  presented 
by  a  case  of  spasmodic  torticollis. 


592 


NKi;\()lS  DISEASES 


The  spasms  consist  of  sudden  quick  contractions  of  the 
affected  muscles,  causing  the  head  to  turn  in  the  direction 
of  action  of  the  muscle.  In  other  cases  it  is  of  a  slower 
and  more  prolonged  character,  by  which  the  head  may  be 
turned  steadily  to  one  or  other  side,  and  maintained  there 
for  an  appreciable  period  before  the  spasm  relaxes. 

On    the  other  hand,  it  may  be  arrested   by  some  simple 

process,  such  as  placing 
the  finger  on  or  near 
the  chin. 

The  symptoms  vary 
according  to  the  muscles 
which  are  affected.  The 
sterno-mastoid  is  most 
often  the  seat  of  spasm, 
but  it  may  be  associated 
with  the  upper  part  of 
the  trapezius  upon  the 
same  side.  The  splenius, 
more  especially  on  the 
opposite  side,  is  less 
commonly  involved. 
The  scaleni,  platysma 
myoides,  and  the  deeper 
neck  muscles  are  only 
rarely  the  seat  of  spasm. 
"Various  types  of 
spasmodic  turning  of 
the  head  are  observed. 
If  the  sterno-mastoid  is  alone  affected,  the  face  is  turned 
to  the  opposite  side,  the  chin  is  protruded,  and  the  head 
inclined  towards  the  same  side  as  the  affected  muscle. 

An  associated  spasm  of  the  upper  part  of  the  trapezius  on 
the  same  side  will  incline  the  head  to  that  side  and  draw  it 
slightly  backwards.  The  simultaneous  action  of  both  trapezii 
and  splenii  results  in  a  backward  movement  of  the  head 
(retro-collic  spasm). 

A  spread  of  the  spasm  to  other  and  adjacent  muscles  is 
not   uncommon.     Thus   the   face,    shoulder,    and   arm   may 
become  affected,  and  in  severe  cases  the  muscles  of  the  back. 
The  spasms  always  cease  during  sleep. 


Fig.  188. — Photograph  of   a  case  of  antero 
collie  spasm. 


THE  TICS  593 

Sensory  symptoms  are  rare.  Pain  of  a  neuralgic  typo 
may  be  complained  of  at  the  back  of  the  head  and  neck.  The 
spasm  itself  is  not  painful,  but  creates  great  discomfort  and 
distress. 

The  course  of  the  disease  varies.  In  some  cases  it  ceases 
either  spontaneously,  or  in  consequence  of  treatment  after 
some  months  or  years.  Some  degree  of  spasm  usually 
persists  in  severe  cases.  It  has  no  influence  upon  the 
duration  of  life. 

Treatment.  The  malady  does  not  lend  itself  satisfactorily 
to  the  influence  of  drugs  ;  and  what  has  been  said  upon  the 
treatment  of  tics  in  general  holds  good  for  that  of  torticollis 
spasm.  The  bromides,  valerian,  belladonna,  trional  and  the 
hypnotics,  cannabis  indica,  and  conium  may  all  be  tried  with 
some  benefit.  In  the  severe  cases,  with  spread  of  the  spasm 
to  the  shoulders,  arms,  and  back,  we  have  seen  much  temporary 
benefit  result  from  hypodermic  injections  of  the  hydrobromate 
of  hyoscine. 

Galvanism  has  been  of  occasional  use  in  some  cases  when 
the  positive  pole  is  applied  over  the  affected  muscles.  In 
other  cases  it  has  apparently  aggravated  the  spasms. 

Hypnotic  suggestion  has  been  of  use  in  cases  which  are 
of  an  hysterical  character. 

Operative  interference  such  as  division  of  the  spinal 
accessory  nerve  may  temporarily  arrest  the  movements  ;  but 
the  only  operation  likely  to  induce  permanent  benefit  consists 
in  dividing  the  spinal  accessory  nerve  on  one  side  and  the 
posterior  primary  divisions  of  the  cervical  nerves  upon  the 
other. 


38 


INDEX 


Abductor  paralysis  of  vocal  cords,  92 
Abdominal  reflexes,  43 
Abscess,  intracranial,  279 

of  spinal  cord,  326 
Acromegaly,  with  pituitary  tumours, 

261 
Acute  ascending  myelitis,  324 
ascending  paralysis,  446 
atrophic    paralysis    of    adults, 

438 
disseminated  myelitis,  324 
labyrinthitis,  66 
meningo-myelitis,  320 
myelitis,  320 
polio-myelitis,  438 
suppurative  myelitis,  326 
toxic  polyneuritis,  147 
transverse  myelitis,  321 
Adductor  paralysis  of  vocal  cords,  92 
Aerophagia,  586 
Afferent  cerebellar  tracts,  19 
Agenesia,  212 
Ageusia,  54 
Akinesia  algera,  546 
Alcoholic  neuritis,  136,  141 

clinical  varieties   of, 

137,  139 
complications  of,  140 
Alexia,  183 

subcortical,  56 
Amaurotic  family  idiocy,  482 
Amyotonia  congenita,  467 
Amyotrophic  lateral  sclerosis,  408, 413 
and  bulbar  paralysis,  416 

chronic  ophthalmoplegia, 
416 
Anaemia    in    subacute    combined   de- 
generation, 430 
Anal  reflex,  48 
Aneurism,  intracranial,  208 

miliary,  211 
Angioneurotic  oedema,  456 
Ankle  clonus,  46 
Ankle  jerk,  46 
Anorexia  nervosa,  539 
Anosmia,  52 

in  frontal  tumours,  245 


Anterior  cerebral  artery,  167 
choroidal  artery,  169 
crural  nerves,  126 
crural  neuralgia,  161 
horns,  nuclei  of,  8 
Antero-lateral  columns,  307 

ascending  tracts  of,  308 
descending  tracts  of,  307 
Aphasia,  180 

examination  of,  185 

in  lesion   of   temporo-sphe- 

noidal  lobe,  183 
Marie's  hypothesis  of,  182 
subcortical,  184 
Apraxia,  186 

in   tumours   of   corpus  cal- 
losum,  261 
Aqueduct  of  Sylvius,  5 
Argyll-Robertson 

phenomenon,  34,  78 

in  general  paralysis,  403 
in  tabes  dorsalis,  387 
pupil,  ciliary  ganglion  in,  78 
Arsenical  neuritis,  141 
Astasia  abasia,  533 
Astereognosis,  251 
Ataxia,  in  cerebellar  lesions,  21,  263 

tabes  dorsalis,  389 
Athetosis,  216,  221 
Atonia,  in  tabes  dorsalis,  389 
Atrophic  paralysis,  acute,  438 

chronic,  407 
sclerosis,  223 
Auditory  centre,  64,  181 

nerve,  tumours  of,  267 
tract,  63 
vertigo,  66 
word-centre,  183 
Audito-psyehical  centres,  65 
Auras  in  epilepsy,  565 
Auto-intoxication  in  epilepsy,  564 
Automatism  in  epilepsy,  566,  569 


B 


Babinski's  sign,  44 

Bacteriologv  of   cerebro-spinal  fluid, 
50 


595 


38 


596 


INDEX 


Basis  oranii,  tumours  of,  272 

Beriberi,  L50 

Betz  cells,  I 

Bed-sores  in  myelitis.  322 

liieeps  jerk,   ltl 

Brachial  neuralgia,  L60 

plexus,  cords  of,  1 1 1 
inner  cord  of,  1 12 

lesions  of,  107 
lesions  of  lower  trunk,  109 
of  middle  trunk,  109 
of  upper  trunk.  108 
outer  cord  of,  11 1 
posterior  cord  of,  1 1  1 
root  lesions  of,  104 
Brachium  conjunctivum,  20 
Brain,   genera]   symptoms  of  lesions 
of,  171 
vascular  supply  of,  166 
Bromism,  oSl 

Brown  -  Sequard's    '  Symptom  -  Com- 
plex,' 15 
in  cerebro-spinal  syphilis,  375 
in     compression     paraplegia, 

353 
in  liamiatomyelia,  342 
in    intra-medullary   tumours, 
339 
Bulbar  arteries,  170 

paralysis,  409,  416 

tongue  in,  93 
vagus  nerve  in,  91 
svmptoms       in       myasthenia 
gravis,  488 
Bulbo-cavemosus  reflex,  44 
Buzzard's  myopathy.  465 


Caisson  disease,  344 
Caries  of  spine,  355 

differential   diagnosis  of, 

360 
in  special  localities,  359 
laminectomy  in,  362 
Cauda  equina,  lesion  of,  119 

differential  diagnosis,  123 
Cavernous  sinus,  171 

thrombosis  of,  287 
Cells  of  Betz,  1 
Centrum  ovale,  2 
Cerebellar  abscess,  284 
arteries.  Kill 
conduction  in  cord,  313 
hemorrhage,  200 
Lesions,  hypotonia  in,  21 

ni,  18 
tracts,  19,20 
tumours,  262 
bello-pontine  t  amours,  267 


Cerebellum,  affections  of,  in  children. 

225 
ohronic  disorders  of,  478 
encephalitis,  226 
experimental  lesions  of,  21 
physiology  of,  21 1 
tumours  of,  262 
vascular  lesions  of,  226 
Cerebral  diplegia.  212 
embolism.  193 

hemorrhage,  L9  I 
paralysis  in  infancy,  21 1 

softening,  189 
tumours  :  see  Tumours,  226 
vessels,  167 
Cerebro-spinal  fluid.  t8 

bacteriology    of, 

50 
cytology  of,  48 
in  cerebro-spinal 

syphilis,  49 
in  epilepsy,  563 
in  general  para- 
lysis, 402, 405 
in    poliomyelitis 

acuta,  439 
in  tabes  dorsalis, 

385 
Wassennan     re- 
action of,  405 
meningitis,  293 
syphilis :  see  Syphilitic 
'  Diseases,  369 
Cervical  ribs,  symptoms  of,  110 
Cervico-occipilal  neuralgia,  160 
Cheilophagia,  586 
Chorea,  500 

hereditary,  483 
insaniens,  503 
tetanoid,  513 
Chronic  atrophic  paralysis,  407 
labyrinthitis,  66 
ophthalmoplegia,  416 
Cilio-spinal  reflex,  34,  47 
Circle  of  Willis,  166 
Circumflex  nerve,  113 
Clarke's  column,  9,  19 
t  'laiulieat  ion.  intermit  tent,  452 
Clonus  of  ankle.  4(5 
Coccygodynia,  162 
Cochlear  ner\  e,  63 
Combined     degeneration     of     spinal 

eonl,  429 
'  Comma  '  tract,  307 
Compression  paraplegia.  :',|ii 
( lonjugate  movements  of  eyes,  79 

paralysis  of,  79 
Conus  mcdullaris,  lesions  of,  123 
Convulsive  tic,  587 
Coprolalia,  586 

Corneo-conjunctival  reflex,  43 
Cornu-radicular  zone,  9 


IN  DUX 


507 


Corona  radiata,  2 

Corpus  dentatum  cerebelli,  18 

Cortical  motor  area,  1 

sensory  area,  LI,  18 
Craft  paralysis,  513 
Cranial  nerves,  70 
Cranio-tabcs,  377 
Cremasteric  reflex,  44 
Crossed  pyramidal  tract,  5 
Crura  cerebri,  symptoms  of  lesions  of, 

172 
Cutaneous     sensibility,    examination 

of,  40 
Cystic  formation,  223 
Cysticercus  cellulosas,  226 
Cytology  of  cerebrospinal  fluid,  48 


I) 


Dairymaid's  cramp,  510 

Decussation  of  pyramids,  5 

Deep  reflexes,  45 

sensibility,  44 

examination  of,  41 

Degeneration,  reaction  of,  51 
stigmata  of,  25 

Deiters's  nucleus,  18 

Diabetic  neuritis,  144 

Diphtheritic  paralysis,  144 

Diplegia,  acquired,  216 
congenital,  212 

Diplopia,  71 

Direct  pyramidal  tract,  5 

Dislocation  of  spine,  364 

Disorders  of  movement,  176 

Disseminated  sclerosis,  418 

clinical  types  of,  425 
differential  diagnosis  table, 

428 
gait  in,  423 

mental  condition  in,  421 
oculomotor  paralysis  in,  422 
optic  atrophy  in,  422 
papillitis  in,  422 
speech  defects  in,  423 
sphincters  in,  425 
tremor  in,  423 
types  of,  425 

Diver's  paralysis,  344 

Dreamy  states,  in  temporo-sphenoidal 
tumours,  255 

Dysphagia,  540 

Dystrophies,  muscular,  458 


E 


Echokinesis,  586 

Echolalia,  586 

Efferent  cerebellar  tracts, 


20 


Eigb i  li  nerve,  ;i nai omy  of,  61 
diseases  of,  65 
physiology  of,  62 
i  amours  of,  207 
Electrical  examination,  50 

read  ions  in  disease,  51 
Embolism  of  central  artery  of  retina, 

60 
Encephalitis,  217 

morbid  anatomy  of,  217 
symptoms  of,  218 
traumatic,  212 
Epicritie  sensibility,  14 
Epidemic    cerebro-spinal    meningitis-. 

300 
Epigastric  reflex,  44 
Epilepsy,  560 

auras  in,  565 
automatism  in,  569 
causes  of,  561 
chemical  pathology  of,  563 
clinical  types  of,  571 
combined  types  of,  571 
course  of,  573 
dementia  in,  570 
differential  diagnosis,  tables 

of,  576 
diurnal,  572 
focal,  177 
frequency  of,  574 
heredity  in,  560 
in  organic  disease,  583 
Jacksonian,  177 
major  fits,  566 

types  of,  571 
minor  fits,  566 

types  of,  571 
nocturnal,  572 
pathology  of,  562 
paroxysmal    psychoses    in, 

569 
prognosis  in,  577 
psychical  condition  in,  568 
psychical  equivalents  of,  570 
pupil  in,  567 
sequela?  of,  567 
serial,  572 

status  epilepticns,  572 
temperament  in,  568 
treatment  of,  578 
Equilibration  tests,  31 
Erb's  juvenile  muscular  atrophy,  463 

paralysis,  108 
Erroneous  projection,  72 
Erythromelalgia,  453 
Evascularisation,  187 
Examination  of  special  senses,  25 

hearing.  29 
smell.  25 
taste.  26- 
vision.  26 
Extensor  plantar  response.  44 


:»ds 


INDKX 


External   cutaneous  uerve,  paralysis 
of,  L27 

popliteal  nerve,  pa  rah  sis  of, 

130 
red  us  muscle,  paralysis  of, 
74 
Extra-cerebellar  tumours,  267 
K\i  ra-dural  abscess,  285 

hemorrhage  of  cord,  364 


F 

Fao  .  in  chorea,  502 

in  myasthenia  gravis,  488 
in  myopathias,  4(34,  4(35 
in  paralysis  agitans,  494 
in  tabes  dorsalis,  387 
Facial  hernial  rophy,  454 

muscular  dystrophy,  4(33 
nerve,  83 

examination  of,  35 
sensory  division  of,  10 
paralysis,  84 

and  mastoid  operation,  89 
electrical  reactions  in,  87 
herpes  zoster  in,  87 
in  hemiplegia,  174 
in  lesions  of  optic  thala- 
mus, 174 
loss  of  taste  in,  86 
myasthenia  gravis,  89 
in  the  myopathies  89 
of  cerebral  origin  88 
of  nuclear  origin,  89 
pathology  of,  84 
peripheral  type,  89 
prognosis  of,  89 
secondary  contracture  in, 

87 
treatment  of,  89 
facial  spasm,  588 

tic,  588 
Falx  cerebri  :  see  Tumours,  259 
Familial  diseases,  458 
Family  periodic  paralysis,  470 

spasl  ic  paralysis,    INI 
Faradic  contractility,  50 
Fasciculus  solitarius,  in 
Fifth  nerve,  80 

examination  of,  34 
herpes  of,  82 
neuralgia  of,  155 
neuro-paralytic    keratitis, 

83 
paralysis  of,  82 
sensory  distribution  of,  80 
nucleus,  6 
Fillet,  accessory,  4 
mesial,  10,  1 7 
lateral,  63 
Fits  :  see  Epilepsy,  566 
see  Hysteria,  524 


Flechsig's  tract,  19 
Focal  epilepsy,  1 77 
Fourth  aerve,  paralysis  of,  76 
nucleus,  (i 

l-'racl  ore  of  spine,  364 
Friedreich's  ataxia,  47 4 

G 

Gait,  in  cerebellar  disease,  2(34 

in  disseminated  sclerosis,  423 

in  hemiplegia,  -i  »7 

in  Huntington's  chorea,  484 

in  myasthenia  gravis,  4S9 
in  paralysis  agitans,  496 
in    pseudo-hypertrophic    para- 
lysis, 460 
in  tabes  dorsalis,  390 
( ralvanic  con!  motility,  50 
( rastric  crises,  393 
General  paralysis,  400 

cerebro-spinaJ    Quid    in, 

402 
clinical  types  of,  405 
cranial  nerves  in,  403 
diagnosis  of,  405 
epileptiform  seizures  in, 

404 
etiology  of,  400 
juvenile  type  of,  406 
mental  condition  in,  402 
pathology  of,  401 
prognosis  of,  406 
pupils  in,  403 
reflexes  in,  404 
speech  in,  404 
symptoms  of,  402 
treatment  of,  406 
tremor  in,  403 
Wasserman  reaction  in, 
405 
Glossopharyngeal  nerve,  7,  10 
paralysis,  91 
Gluteal  paralysis,  44 
Gowers's  tract,  17 
Great  scial  ic  aerve,  128 
Gumma  of  brain,  :$72 
Gumma  of  spinal  cord,  377 


II 


Habit  spasm,  584 
Hsematomyelia,  340 

differential     diagnosis 

of,  342 
in  syringomyelia,  340 
Hearing  in  extracerebellar  tumours, 
268 
and  equilibration,  61 
Heart  in  chorea,  504 

in  vagal  affections,  92 


IN  DUX 


599 


Eelweg's  bundle,  309 
I  [emianopsia,  28 

bitemporal,  (iJ 

heteronymous,  28 
homonymous,  28,  57 
nasal,  61 

pupillary  reaction,  34 
quadrantic,  58 
Hemiatrophy  of  face,  454 
Hemichorea,  502 
Hemiplegia,  173,  206 

abdominal    muscles    in, 

174 
associated      movements 

in,  174 
character     of     paralysis 

in,  176 
disorders    of    movement 

in,  176 
distribution  of  paralysis 

in,  174 
facial  paralysis  in,  174 
gait  in,  207 

hemianaesthesia  in,  174 
hemianopsia  in,  174 
infantile,  216 

athetosis  in,  221 
causes  of,  216 
convulsive     seizures 

in,  222 
cranial  nerves  in,  218 
disorders   of    move- 
ment in,  220 
face  in,  218 
gait  in,  219 
motor  system  in,  218 
reflexes  in,  219 
sensation  in,  219 
speech  in,  219 
trophic    changes  in, 
219 
mental  condition  in,  207 
muscular  tone  in,  176 
paralysis  in,  206 

of    bilateral    move- 
ments in,  173 
of  conjugate  lateral 
movements  in,  173 
of  homolateral  mus- 
cles in,  173 
recovery  in,  174,  206 
reflexes  in,  177,  207 
respiratory     movements 

in,  173 
rigidity  in,  174 
sensation  in,  176 
spasticity  in,  176 
tongue  in,  93,  174 
treatment  of,  208 
trophic  changes  in,  176 
vasomotor    changes    in, 
176 


Eemorrhage,  cerebellar,  200 

ingravescent,  \'.)H 
meningeal,  200 
pontine,  L99 
ventricular,  \'.)H 
I  [ereditary  cerebellar  ataxia,  4/78 

chorea  :  see  II  nut  ington's 

chorea,  483 
spastic  paraplegia,  481 
Eerpes  zoster,  162 

of  fifth  nerve,  82 

I  lelrnmvmons  hemianopsia,  28 
Eigher  visual  centres,  56 
Huntington's  chorea,  483 

psychical     symptoms 

in,  484 
speech  in,  484 
Hydrocephalus,  288 

etiology,  289 
congenital,  289 
primary,  290 
prognosis  in,  291 
secondary,  290 
treatment,  292 
Hydromyelia,  327 

Hypertrophic  cervical  meningitis,  376 
Hypertrophy  of  pons,  270 
Hypoglossal  nerve,  36,  93 

in  bulbar  palsy,  93 
in  hemiplegia,  93 
in  syringomyelia, 

93 
in  tabes  dorsalis, 

93 
paralysis  of,  93 
Hysteria,  522 

affections  of  speech  in,  529 
air-swallowing  in,  539 
anorexia  nervosa,  539 
automatism  in,  526 
blindness  in,  528 
cardiovascular    system    in, 

542 
colour  vision  in,  528 
contractures  in,  532 
cranial  nerve,  affections  in, 

527 
deafness  in,  528 
diagnosis  of,  544 
dissociation    of    conscious- 
ness in,  524 
dysphagia  in,  540 
facial  spasin  in,  529 
flaccid  palsy  in,  530 
flatulence  in,  539 
globus  hystericus  in.  525 
hypnotism  in.  527.  546 
joints  in,  541 
lingual  spasm  in,  529 
major.  525 
mental  state  in.  524 
minor,  524 


600 


INDEX 


1 1  j  steria — continued 

ocular  palsies  in,  528 

paral\  .-is  in,  530 
prognosis  in,  544 
reflexes  in.  ."wis 
respiratory  sj  stem,  5  l<  I 
retention  of  urine  in,  539 
seizures  in,  52-4 
sensory  loss  in,  535,  537 
spastic  paralysis  in,  531 
spine,  5-40 
si  apor  in,  527 
subjective  sensations  in,  535 
temperament  in,  523 
trance  in,  527 
treatment  of,  544 
tremors  in,  534 
vasomotor  system  in,  5  12 
visual  fields  in.  527 
vomiting  in,  539 
wind-sucking  in,  539 

Hystero-epilepsy,  526 

Hystero-genetic  zones,  535 


I ut; i mile  paralysis,  438 
Infective  myelitis,  320 
Inferior  longitudinal  sinus,  171 
oblique  muscle,  75 
petrosal  sinus,  171 
rectus  muscle,  74 
Ingravescent  hemorrhage,  198 
Intercostal  neuralgia,  161 
[ntermedio-lateral  horn,  8 
Intermittent  claudication,  452 
Internal  capsule,  2 

popliteal  nerve,  129 
rectus  muscle,  73 
Intracranial  abscess,  284 

cerebellar,  284 
cerebro-spinal  fluid  in. 

283 
diagnosis  of,  284,  288 
emaciation  in,  283 
extradural,  285 
headache  in,  282 
idiopathic,  280 
intradural,  285 
mental  symptoms  in, 

282 
modes  of  infection  in, 

279 
optic  neuritis  in,  282 
pathology  of,  281 
prognosis  in,  284 
pulse  in,  282 
temperature  in,  282 
temporo-  sphenoida  1 , 

283 
vomiting  in,  282 


Intracranial  aneurism,  208 

treatment,  21(1 
t  amours,  226 

Cerebral    pressure   in, 
general  effects  of,  23-1 

intracranial     tension 
in,  233 
\  enOUS  system,  170 
Intradural  abscess,  285 
Isolat  ion  t  real  men! ,  5  15 


.law  jerk,  46 
Jaeger  test-types,  27 

Juvenile  general  paralysis,  406 
tabes  dorsalis,  399 


K 

Klumpke's  paralysis,  109 
Knee  clonus,  46 
jerk,  46 


Labyrinthitis,  acute,  66 

chronic,  66 
Labyrinthine  deafness,  65 

vertigo,  66,  67 
Landouzy-Dejerine,  myopathy,  463 
Landry's  paralysis,  446 
Larynx,  nerve  supply  of,  91 
Laryngeal  crises  in  tabes  dorsalis.  393 
paralysis,  91 

in  hysteria,  529 
in  tabes  dorsalis,  388 
Lateral  fillet,  63 

sinus,  thrombosis  of,  1  7 1 
ventrical  tumours  of,  257 
Lead  neuritis,  143 
Leprosy,  148 
Leprous  neuritis,  148 
Light-reflex  of  pupil,  77 
Lissauer,  zone  of,  \i 
Lit  lie's  disease,  213 
Localisation     of     function     in     spinal 

cord,  311 
Locomotor  ataxy :  nee  Tabes  Dorsalis, 

379 
Lordosis    in    pseudo-hypertrophio 

paralysis,  462 
Lumbar  puncture,  48,  202 

plexus,  126 
Luinbo-sacral  plexus,  lesions  of,  124 
Lymphocytosis  of  cerebro-spinal  fluid, 

49 
Lower  motor  neurone,  5 


INDEX 


601 


M 


Macula,  cortical  representation  of,  57 

Maniacal  chorea,  503 

Mania-m.elanch.olia,  551 

Mario  on  aphasia,  182 

Mastodynia,  162 

Mastoid    upend  ion,  i": i ( - i : 1 1    |>al  ;\    ;i  Pin-, 

89 
Mediastinal  growths,  vagus  in,  91 
Membranes  spinal,  tumours  of,  348 
Meniere's  disease,  65 

diagnosis  of,  68 
pathology  of,  65 
symptoms  of,  66 
treatment  of,  68 
symptom-complex,  65 
Meningeal  hemorrhage,  201 

non-traumatic,  196 
traumatic,  201 
Meningitis,  cerebro-spinal,  293 
blood  in,  296 
etiology  of,  293 
fluid  in,  296 
Kernig's  sign  in,  295 
cervical  hypertrophic :  see 
Syphilitic  Disease,  376 
post-basic,  293,  298 
suppurative,  302 
tuberculous,  299 

cerebro-spinal      fluid 
in,  301 
Meningococcus,  294 
Meralgia  paraesthetica,  148 
Mesial  fillet,  17 
Microgyria,  224 
Midbrain,  tumours  of,  256 
Middle  cerebral  artery,  67 
Migraine,  515 

types  of,  518 
Miliary  aneurism,  211 
Molluscum  fibrosum,  134 
Morvan's  disease,  335 
Motor  aphasia,  182-184 

characters  of,  184 
lesions  in,  184 
Motor  areas,  cortical,  1 

conduction  in  cord,  312 
neurone  (lower),  5 
system,  1 

examination  of,  36 
tics,  586 
Multiple  cerebro-spinal  sclerosis  :  see 

Disseminated  Sclerosis,  418 
Multiple  neuritis,  135 
Muscular    atrophy :    see    Progressive 
Muscular  Atrophy  409 

progressive,  407 
spinal   type   in  chil- 
dren, 471 
dystrophies,  458 

Buzzard's  type,  464 


Muscular  dystrophies     continued 
distal  i  \  pe,  465 
facial,  463 

facio  -  scapulo  -  hume- 
ral, 463 
generalised,  462 
juvenile,  463 
pelvic  type,  I'll 
pseudo  -  hypertrophic 
muscular  para! 
459 
shoulder  girdle  type, 
462 
Myasthenia  gravis,  487 

bulbar    symptoms     in, 

488 
diplopia  in,  488 
electrical  reactions  in, 

489 
facial  expression  in,  488 
gait  in,  489 

ophthalmoplegia  in,  488 
pregnancy  in,  490 
ptosis  in,  487 
reflexes  in,  490 
speech  in,  486 
thymus  gland  in,  486 
Mydriasis,  34 
Myelitis,  302,  320 
acute,  320 

ascending,  324 
cerebro-spinal  fluid  in, 

321 
pathology  of,  320 
suppurative,  326 
transverse,  321 
types  of,  321 
infective,  320 
syphilitic,  374 
Myopathies :    see    Muscular    Dystro- 
phies, 458 
Myosis,  34 

Myotonia  atrophica,  470 
congenita,  469 
'  Myriat  chit,'  588 

N 

Nerve  deafness,  65 
tumours,  135 
Neuralgia,  152 

miscellaneous  forms,  160 
trigeminal,  155 
Neurasthenia,  547 

clinical  types  of,  550 
etiology,  547 
differential     diagnosis 

of,  551 
heredity  in,  547 
prognosis  in,  552 
psychical  state  in,  548 
traumatic.  548 


GOi> 


INDEX 


Neuro-paralytic  keratitis,  s_ 
Neuroses  (occupation  >.  51  19 
X in t  li  in  r\  e,  35 
Nucleus  ambiguus,  1.  7 
oculo-motor,  6 
of  anterior  horns,  8 
of  Deiters,  is 
of  fifth  nerve,  6 
of  fourth  uerve,  ii 
cif  seven!  li  nen  e,  6 
of  sixth  iM'i\  e,  (> 
of  i  bird  urn  e,  5 
of  twelfth  ner\  e,  8 
N  \  stagmus,  7!'.  263 

causes  of,  80 
cerebellar,  80,  263 

i genital,  NO 

in  disseminated  sclerosis, 

80,  422 
in    Friedreich's    disease, 

80,  476 
sci  n  ic  irri  ilar  canals,  32,80 
spontaneous,  80 
strain,  ocular,  80 
vestibular,  32 


0 


( >bturator  nerve,  1-7 

( Iccipital  lobe,  connections  of,  56 

tumours  of,  -'>'■'< 
Occupation  neuroses,  506 
Ocular  motor  nuclei,  6 

paralysis,      in       disseminated 
sclerosis,  422 
in   myasthenia    gravis, 

486 
in  tabes  dorsalis,  388 
tests  for,  33 
vertigo,  72 
Oculo-motor  nerves  : 

congenital    paralysis, 

70 
disease  of,  70 
etiology  of,  70 
hysterical      paralysis 

of,  71,  528 
intra-orbital  paralysis 

of,  70 
nuclear    paralysis   of, 

70,  416 
paralysis    of    central 
origin,  70 
conjugate  move- 
ment, 79 
external     rectus, 

71 
fourth  nerve,   /•> 
inferior    oblique 

muscle,  75 
inferior     rectus, 
muscle,  7  I 


Ooulo-motor   nerves,   paralysis     con- 
fin  a,  ,i 

internal       rectus 

muscle,  78 
si\t  h  ner\  e,  76 
superior    oblique 

muscle,  75 

superior       reotus 

musole,  74 
symptoms  of,  72 
t  bird  nerve,  7ii 
recurring  paralysis  of, 

7(i 
Olfactory  bulb,  52 

cent  res,  52 
tract,  52 
Olivo-cerebellar  bundle,  2(  I 
( iplit  1 1 : i  bnoplegia  chronica,  H6 
( Ippenheim's  reflex,  L5 
Optic  atrophy.  28,  59 

causes  of,  59 
primary,  59 
secondary,  60 
varieties  of,  ."><) 
cliiasma,  (iO 
nerve,  54,  58 
neuritis,  28,  58 

appearances  of,  59 
blindness  in,  59 
causes  of,  58 
in  intracranial  aneur- 
ism, 209 
light-reflex  in,  59 
symptoms  of,  59 

t  real  liient   of,  59 

radiations,  55 
tracts,  54,  60 

diagnosis  of  lesion  of,  61 
Organic  reflexes,  examination  of,  17 

P 

Pachymeningitis     cervicalis     hyper- 
t  rophica,  376 

syphilil  tea,  372 
Pains,  in  tabes  dorsalis.  390 
Palatal  reflex,   1  I 
Paradoxical  plantar  reflex,    |."> 
Parageusia,  5  I 
Paragraphia,  183 
Paralyl  ic     dementia  :     see     General 

Paralysis,  400 
Paralysis,  acute  ascending,  446 
bulbar,  416 

cerebral,  in  infancy,  21(3 
causes  of,  211 
physical      confor- 
mat  ion    in,  224 
secondary  degen- 
erat  ion  in,  224 
hysterical,  530 
of  soft  palate,  92 


INDUX 


603 


Paralysis  agitans,  493 
Paramyoclonus  multiplex,  491 

Paraphasia,  1  82 
Parasyphilitic  diseases,  379 
Parietal  lobe  :  see  Tumours  of,  250 
Parkinson's     disease:     see    Paralysis 

Agitans,  493 
Parosmia,  52 
Pelvic  myopathy,  464 
Perforating  ulcer,  395 
Peripheral  motor  system,  5 

nerves,  anatomy  of,  96 

circumflex  paralysis,  110 
differential    diagnosis    of, 

99 
lesions  of,  97 

prognosis  of  lesions  of,  100 
symptoms  of  lesions  of,  98 
treatment   of    lesions    of, 

101 
long  thoracicparalysis,  103 
median  paralysis,  116 
musculo -spiral    paralysis, 

110 
phrenic  paralysis,  102 
syphilis  of,  135 
ulnar  paralysis,  117 
nervous  system,  96 
neuritis,  135 
Peroneal  muscular  atrophy,  472 
'  Petit  mal,'  566 
Pharyngeal  reflex,  44 
Pharynx,  35 

anaesthesia  of,  91 
Physiology  of  sensory  system,  13 
Pianist's  cramp,  510 
Pick's  bundle,  4 
Pituitary  tumours,  261 
Plantar  reflex,  44 

extensor  reponse,  44 
Poliomyelitis  acuta,  438 
Polymorpho-nuclear  cells,  49 
Polyneuritis,  135 
Pons,  glioma  of,  270 

hemorrhage  into,  199 
hypertrophy  of,  270 
sensory  conduction  in,  17 
tubercle  of,  272 
Ponto-spinal  bundle,  4 
Porencephaly,  212 
Post-basic  meningitis,  293,  298 
Post-central  area,  sensory  representa- 
tion in,  250 
Post-diphtheritic  neuritis,  145 
Posterior  cerebral  artery,  168 
columns  of  cord,  306 

in  tabes,  381 
longitudinal  bundle,  6 
root  ganglion,  9 

in    herpes    zoster, 

162 
in  tabes,  384 


I'rcccnl  nil  area,  2 

Pregnancy  in  disseminated  sclerosis, 
429 

myasthenia  gravis,  490 
Primary  deviation,  72 
Progressive  hypertrophic  interstitial 
neuritis,  151 
muscular  atrophy,  407 

spinal  accessory 
nerve  in,  94 
Protopathic  sensibility,  14 
Pseudo-hypertrophic  muscular  para- 
lysis, 459 
Psychasthenia,  555 
Psychical  tic,  584 
Psycho-motor  area,  2 
Pudendal  plexus,  131 
Pupil,  ciliary  ganglion,  78 
dilatory  fibres  of,  78 
in  general  paralysis,  403 
in  syringomyelia,  331 
in  tabes  dorsalis,  386 
reaction  on  convergence,  78 
Pupillary  light-reflex,  77 

reactions,  34 
Pupils,  33 

Argyll-Robertson       phenome- 
non, 78 
Pyramidal  system,  1 
tracts,  5 


R 


Raynaud's  disease,  449 

complications  of,  451 
stages  of,  449 
Reaction  of  degeneration,  51 
Rectal  crises  in  tabes  dorsalis,  393 
Rectal  reflex,  47 
Recurrent  laryngeal  nerve,  91 
Reflexes,  abdominal,  43 

anal,  44,  48 

bulbo-cavernosus,  44 

cilio-spinal,  47 

corneo-conjunctival,  43 

cremasteric,  44 

deep,  45 

epigastric,  44 

examination  of,  42 

gluteal,  44 

organic,  47 

palatal,  44 

pharyngeal,  44 

plantar.  44 

rectal,  47 

scapular,  44 

superficial,  43 

uterine,  47 

vesical,  47 
Rigidity  in  paralysis  agitans,  495 
Rinne's  test,  30 


604 


INDEX 


Rolando,  fissure  of,  1 

substance  of.  |o 
Romberg's  sign,  31 
Rubro-spinal  bundle,  1.  31 ls 


Sacral  plexus.  127 

St.  Virus's  Dance  :   see  Chorea,  500 

Scapular  reflex,  44,  4-6 

Sciatica,  L57 

differentia]  diagnosis  of,  159 
neuritis.  l.~>7 
Sohultze's  tract,  307 
Schwabach's  test,  30 
Scleroderma,  457 
Sclerose  en  plaques:  see  Disseminated 

Sclerosis,    lis 
Sclerosis,  disseminated  :  see  Dissemi- 
nated sclerosis,  -1 18 
Scotoma,     visual,      in      disseminated 
sclerosis,  421 
in  migraine,  51  7 
Scrotal  reflex,  47 
Secondary  deviation,  72 
Sensation,  cortical  centres  for,  11,  250 
Sensibility,  localization  of,  42 
Sensory  aphasia,  IS2 
system,  9 

cortical  centres,  11,  250 
Seventh  nerve,  35 
nucleus,  6 
Sexual  reflex,  47 
Sigmoid  sinus,  287 
Sinus  thrombosis,  infective,  286 
cavernous,  287 
sigmoid,  287 

superior  longitudinal,  287 
Sixth  nucleus,  6 

nerve,  paralysis  of,  76 
Skew  deviation,  200,  262 
Smell,  examination  of,  25 

loss  of,  52 
Smile  in  myasthenia  gravis,  488 

myopat  hies.  465 
Snellen's  test-types,  26 
Soft    palate,  35 

paralysis  of,  H2 

Spastic  paraplegia,  hereditary.   IS| 

Special  senses,  52 

Speech,  development  of,  180 

in  disseminated  sclerosis,  423 
in  Friedreich's  disease,  476 
in  general  paralysis,  404 
in  Huntington's  chorea,  484 
in  infantile  cerebral  paralysis, 

216,  219 
in  myasthenia  gravis,  488 
in  paralysis  agitans,  494 
motor  aphasia,  182 

mechanism  of,  181 


speech     continued 

sensor\   aphasia,   L82 

mechanism  of,  181 
Spinal  accessory   nerve,  94 
apoplexy  .  340 

caries,    abscess    formation    in, 
360 
cervico-dorsal  region,  359 
compression  of  cord  in,  356 
cord  symptoms  in,  35s 
laminectomy  in,  362 
lumbar  region,  359 
occipito-al  lo-axoid,  359 
pa!  hologv  of,  355 
root  symptoms  in,  357 
spine  in,  359 

upper  cervical  region,  359 
cord,  abscess  of,  326 
anatomy  of,  305 
cavities  in.  327 
compression  of,  3  16, 
grey  matter  of,  305 
hemorrhage  into,  340 
injuries  of,  364 

extradural    hemor- 
rhage, 364 
fracture    -   disloca- 
tion, 364 
subacute    combined    de- 
generation of,  429 
tracts  of,  306 
tumours  of,  336 
gliosis,  327 
membranes,  348 
Spine,  carcinoma  of,  362 
injuries  of,  364 
malignant  disease  of,  362 

cord  symptoms 

in,  363 
root  symptoms 
in,  363 
sarcoma  of,  362 
Spinocerebellar  tract,  19 

tracts,     degeneration 
of,  480 
Spinothalamic  system,  10,  17 
Status  epilepticus,  572 
Stereognostic  sense,  42,  251 
Sternomastoid  muscle,  94 
Stigmata  of  degeneration,  25 
Strabismus,  72 

convergent,  72 
divergent,  72 
paralytic,  72 
true,  72 
Straight  sinus,  171 
Subacute   combined   degeneration   of 
cord,  429 
anaemia  in,  430 

hemorrhage  from  bladder  in,  435 
pathology  of,  430 
reflexes  in,  434 


INDEX 


(if).', 


Subacute  combined  degeneration   of 
cord — continued 
sensation  in,  434 
stages  of,  434 
types  of,  43G 
Superficial  reflexes,  43 
Superior  laryngeal  nerve,  91 
Superior  longitudinal  sinus,  170,  287 
oblique,  paralysis  of,  75 
rectus,  paralysis  of,  74 
Supinator  jerk,  46 
Suppurative  meningitis,  302 
Swallowing,  47 
Sydenham's  chorea,  500 
Sylvius,  aqueduct  of,  5 
Symmetrical  gangrene,  449 
Syphilis  of  nervous  system,  369 
Syphilitic  diseases  of  nervous  system  : 
cerebro-spinal  fluid,  in,  373 
cerebro- spinal  lesions,  370 
prognosis  of,  373 
treatment  of,  378 
varieties  of,  370 
Syphilitic  gummata,  372 

lepto-meningitis,  371 
lesions  (diffuse),  373 
myelitis,  374 
pachymeningitis,  372 
sclerosis  of  brain,  377 
vascular  lesions,  370 
Syringomyelia,  327 

cervical    sympathetic 

in,  331 
cranial  nerves  in,  330 
diagnosis  of,  335 
pathology  of,  327 
sensory  symptoms  in, 

332 
trophic     disturbances 
in,  334 


T 


Tabes  dorsalis,  379 

arthropathies  in,  385,  393 
ataxia  in,  389 
atonia  in,  389 
blood-vessels  in,  385 
cerebro-spinal  fluid  in,  385 
ciliary  ganglion  in,  385 
clinical  types  of,  396 
cranial  nerves  in,  386 
degenerations  in,  382 
emaciation  in,  396 
etiology  of,  380 
face  in,  388 

Fraenkel's  exercises  in,  399 
gait  in,  390 
juvenile  type  of,  399 
mental  condition  in,  389 
micturition  in,  392 


Tabes  dorsalis — continued, 

morbid  anatomy  of,  381 
ocular  paralysis  in,  388 
optic  atrophy  in,  38  I 

|>orforal  ing  ulcfrs  in,  395 
peripheral  nerves  in,  384 
posterior  roots  in,  384 

root  ganglia    in, 
384 
prognosis  in,  394 
pupils  in,  386 
reflexes  in,  392 
Eomberg's  sign  in,  390 
sensory  symptoms  in,  391 
sexual  functions  in,  393 
treatment  of,  397 
trophic  changes  in,  393 
visceral  crises  in,  393 
vocal  cords  in,  388 
Wasserman  reaction  in,  405 
Tailor's  cramp,  510 
Taste,  cortical  centres  of,  53 
course  of  fibres  of,  53 
examination  of,  26 
in  facial  paralysis,  86 
in  glossopharyngeal  paralysis,  91 
Temporo-sphenoidal  abscess,  283 

lobe, tumours  of : 
see    Tumours, 
254 
Tendon  reflexes  :  see  Deep  Reflexes,  45 
Tenth  nerve,  35 
Tetanoid  chorea,  513 
Tetany,  507 

Thalamo-cortical  system,  9,  11 
Thalamo-spinal  bundle,  4 
Third  nerve  nucleus,  5 

paralysis  of,  76 
Thomsen's  disease,  469 
Thrombosis  of  central  retinal  artery, 
60 
cerebral:    see  Vascular  Lesions, 

188 
in  infancy,  211 
Thymus  gland,  in  epilepsy,  563 

in    myasthenia,    485, 
486 
Tic  douloureux,  155 
Tics,  585 

causes  of,  585 
convulsive,  587 
diagnosis  of,  588 
prognosis  in,  589 
treatment  of,  589 
varieties  of,  5S6 
Tinnitus  aurium,  67 
Tongue,  wasting  of,  93 
Torcular  Herophili,  171 
Torticollis  tic,  590 

Toxic  degeneration  of  lower  neurone, 
148 
polyneuritis,  acute,  147 


606 


INDEX 


Trance,  in  hysteria,  527 
Transverse  myelitis,  acute,  321 
Traumatic-neurasthenia,  548 
Tremor,  in  frontal  tumours,  245 
in  hysteria,  534 
in  paralysis  agitans,  496 
Triceps  jerk,  46 
Trigeminal  nerve,  paralysis  of,  SO 

sensory  root  of,  LO 
neuralgia,  155 
Trophic  diseases,  1  1!» 
Tuberculous  meningitis,  299 
Tumours,  intracranial.  226 

anatomical  factors  in, 

230 
basis  cranii,  272 
cause  of  death  in,  240 
cerebellar,  202 
cerebral       circnl.u  ion 

in,  233 
corpus  callosum,  260 
cysticercus,  2G6 
diagnosis  of,  274 
extra-cerebellar,  267 
falx  cerebri,  259 
fourth  ventricle,  265 
frontal  lobe,  243 
lateral  ventricle,  257 
midbrain,  256 
motor  area,  246 
occipital  lobe,  253 
operation  in,  277 
post-central  and  pari- 
etal, 250 
pre-central,  247 
pituitary,  261 
pressure  cone  in,  237 
pontine,  271 
situation  of,  229 
superior  temporal,  255 
subtentorial,  238 
symptoms  of,  241 1 
temporo  -  sphenoidal, 

254 
third  ventricle,  258 
treatment  of,  276 
uncinate  lobe,  255 
vermis  cerebelli,  265 
varieties  of,  227 
of  peripheral  nerves,  132 
of  spinal  cord,  156 

extra-medul- 
lary, 348 
intra-medul- 
larv,  336 
Twelfth  nerve,  36 
nucleus.  S 


u 


Upper  motor  neurone,  1 
Uterine  reflex,  47 


V 


Vagal  al  lacks.  577 

\'a^o-e-losso]iharynu-eal  nerve.  10,  35 
VagUS  nerve,  paralysis  of,  90 

symptoms  of  paralysis 

'  of,  in 
Vascular  lesions.  I  SO 

differential  diagnosis  of, 

204,  205 
embolism,  193 

causes  of,   193 
prognosis  of,  193 
symptoms  of,  L93 
temperature  in,  L93 
treatment  in,  L94 
etioloL!\    of,   ISO 
hemorrhage,   causes  of, 
1 94 
cerebellar,  200 
compression  in,  L98 
death  in,  196 
effects  of,  196 
etiology  of,  197 
loss    of    conscious- 
ness in,  197 
ingravescent,  198 
intracranial      pres- 
sure in,  196 
meningeal,  196 
mode    of  onset   of, 

197 
operation  for,  203 
pontine,  199 
premonitory  symp- 
toms of,  197 
prognosis  in,  201 
situations  of,  195 
symptoms  of,  197 
t  treatment  of,  202 
ventricular,  198 
in     infancy,    embolism, 
212" 
hemorrhage,  212 
thrombosis,  211 
intracrania  I      aneurism, 

208 
methods  of  production, 

187 
miliary  aneurism,  211 
partial  occlusion,  187 
syphilitic,  371 
i  hrombosis,  188 

premonitory  symp- 
toms of,  190 
prognosis  in,  191 
symptoms  of,  190 
treatment  of,  192 
supply  of  brain,  166 
Vaso-motor  diseases,  449 
Veins  of  Galen,  171 
Venesection,  202 


INDEX 


607 


Ventra]  spino-cerebellar  bract,  lit 
Ventricular  hemorrhage,  l!)8 
Vermis  cerebe]H,  tumours  of,  265 
Vertigo,  in  tumours  of  pons,  270 

in  labyrinthine  disease,  66 
in  ocular  disease,  72 
Vesical  reflex,  47 
Vestibular  apparatus,  131 
nerve,  19 

anatomy  of,  61 
connections  of,  61 
physiology  of,  62 
nystagmus,  32 
Vestibulo-spinal  tract,  20 
Violinist's  cramp,  510 
Visceral  sensibility,  14 
Vision,  anatomy  of,  54 

cortical  centres  of,  56,  181 
examination  of,  26 
physiology  of,  57 
Visual  fibres,  55 

fields,  28,  58 
word-centre,  183 
Vocal  cords  in  tabes  dorsalis,  388 


Von  Recklinghausen     di  ea  e,  L3  l 


w 


Wasserman  reaction  in  general  paral 
sis,  405 
in    syphilitic    di- 
seases, 'M'-', 
in  tabes  dorsalis, 
406 
Weber's  test,  30 

Werdnig-Hoffmann's  paralysis,  409 
Werner's  artificial  memory,  75 
Wernicke's  area,  56 
Word  deafness,  65,  182 
Writer's  cramp,  570 
Wry-neck,  95 


/. 


Zone  of  Lissauer,  9 


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LONDON  AND  ETON 


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DATE   DUE 

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AY  22  1911 

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